polymyositis

What is polymyositis?

Polymyositis is a disease of muscle featuring inflammation of the muscle fibers. The cause of the disease is not known. It begins when white blood cells, the immune cells of inflammation, spontaneously invade muscles. The muscles affected are typically those closest to the trunk or torso. This results in weakness that can be severe. Polymyositis is a chronic illness with periods of increased symptoms, called flares or relapses, and minimal or no symptoms, known as remissions.

Polymyositis is slightly more common in females. It affects all age groups, although its onset is most common in middle childhood and in the 20s. Polymyositis occurs throughout the world. Polymyositis can be associated with skin rash and is then referred to as "dermatomyositis." It also can affect other areas of the body and is, therefore, referred to as a systemic illness. Occasionally, it is associated with cancer or with other diseases of connective tissue (see systemic lupus erythematosus, scleroderma and rheumatoid arthritis).

What causes polymyositis?

To date, no cause of polymyositis has been isolated by scientific researchers. There are indicators of heredity (genetic) susceptibility that can be found in some patients. There is indirect evidence of infection by a virus that has yet to be identified in a form of polymyositis that is particularly resistant to treatment, called inclusion body myositis. This form of polymyositis is diagnosed by the pathologist, a physician specialist who interprets the microscope findings of muscle tissue. The muscle tissue in this form of polymyositis displays clear areas within the muscle cells (called vacuoles) when viewed under the magnification of a microscope.

Researchers from Sweden at the national meeting of the American College of Rheumatology in 2007 reported their findings that T-cells of the immune system in some polymyositis or dermatomyositis patients reacted against cytomegalovirus (CMV) and that detectable antibodies against CMV were present. Their conclusion was that there may be subsets of patients who develop their disease, in part, because of infection with this particular virus.

Aside from diseases with which polymyositis can be associated (as mentioned above), many other diseases and conditions can mimic polymyositis. These include nerve-muscle diseases (such as muscular dystrophies), drug toxins (such as alcohol, cocaine, steroids, colchicine, hydroxychloroquine, and cholesterol-lowering drugs, called statins), metabolic disorders (where muscle cells are unable to process chemicals normally), hormone disorders (such as abnormal thyroid), calcium and magnesium conditions, and infectious diseases (such as influenza virus, AIDS, streptococcus and Lyme bacteria, pork tapeworm and schistosomiasis).

Complications

Possible complications of polymyositis include:

• Difficulty swallowing. If the muscles in your esophagus are affected, you may have problems swallowing (dysphagia), which in turn may cause weight loss and malnutrition.
• Aspiration and pneumonia. Difficulty swallowing may also lead to entrance of food or liquids, including saliva, into your lungs (aspiration), which can lead to pneumonia.
• Breathing problems. If your chest muscles are affected by the disease, you may experience breathing problems, such as shortness of breath or in severe cases, respiratory failure.
• Calcium deposits. Late in the disease, particularly if you've had the disease for a long time, deposits of calcium can occur in your muscles, skin and connective tissues (calcinosis).

Associated conditions
Although these are not complications, polymyositis is often associated with other conditions that may cause further complications of their own, or in combination with polymyositis symptoms. Associated conditions include:

• Raynaud's phenomenon. This is a condition in which your fingers, toes, cheeks, nose and ears turn pale when exposed to cold temperatures.
• Other connective tissue diseases. Other conditions, such as lupus, rheumatoid arthritis, scleroderma and Sjogren's syndrome, can occur in combination with polymyositis.
• Cardiovascular disease. Polymyositis may cause the muscle of your heart to become inflamed (myocarditis). In a small number of people who have polymyositis, congestive heart failure and heart arrhythmias may develop.
• Lung disease. A condition called interstitial lung disease may occur with polymyositis. Interstitial lung disease refers to a group of disorders that cause scarring (fibrosis) of lung tissue, making lungs stiff and inelastic. Signs and symptoms include a dry cough and shortness of breath.

Tests and diagnosis

Diagnosis of polymyositis isn't always easy and can be a lengthy process. Even though the attempt to diagnose your condition may be frustrating, remember that an accurate diagnosis is necessary to receive appropriate treatment.

In addition to a thorough physical exam, your doctor will likely use other tests to confirm a diagnosis of polymyositis.

Imaging tests

• Magnetic resonance imaging (MRI). A scanner creates cross-sectional images of your muscles from data generated by a powerful magnetic field and radio waves.

  As MRI has become more sensitive, doctors have been using it more to diagnose inflammatory myopathies. MRI can detect active inflammation in your muscles, fibrosis and calcification. Its high sensitivity can detect subtle muscle inflammation and swelling early in the disease. A benefit of MRI is that it can look at large amounts of muscle to look for patterns or patches of muscle weakness, instead of taking a small sample from a single muscle, for example.

Muscle tests

• Electromyography. A doctor with specialized training inserts a thin needle electrode through the skin into the muscle to be tested. Electrical activity is measured as you relax or tighten the muscle, and changes in the pattern of electrical activity can confirm a muscle disease. The doctor can determine the distribution of the disease by testing different muscles.
• Muscle biopsy. A small piece of muscle tissue is removed surgically for laboratory analysis. A muscle biopsy may reveal abnormalities in your muscles, such as inflammation, damage or infection. The tissue sample can also be examined for the presence of abnormal proteins and checked for enzyme deficiencies. In polymyositis, a muscle biopsy typically shows inflammation, dead muscle cells (necrosis), and degeneration and regeneration of muscle fibers.

Blood tests

• Blood analysis. A blood test will let your doctor know if you have elevated levels of muscle enzymes, such as creatine kinase (CK) and aldolase. Increased CK and aldolase levels can indicate muscle damage. A blood test can also detect specific autoantibodies associated with different symptoms of polymyositis, which can help in determining the best medication and treatment.

Treatments and drugs

Although there's no cure for polymyositis, treatment can improve your muscle strength and function. The earlier treatment is started in the course of polymyositis, the more effective it is, leading to fewer complications.

Drugs

• Corticosteroids. These medications suppress your immune system, limiting the production of antibodies and reducing muscle inflammation, as well as improving muscle strength and function. Corticosteroids, especially prednisone, are usually the first choice in treating inflammatory myopathies such as polymyositis.

  Your doctor may start with a very high dose, and then decrease it as your signs and symptoms improve. Improvement generally takes about two to four weeks, but therapy is often needed for years.

  Prolonged use of corticosteroids can have serious and wide-ranging side effects, so your doctor may recommend supplements to combat them, such as calcium and vitamin D, and may prescribe bisphosphonates, such as alendronate (Fosamax), risedronate (Actonel) or zoledronic acid (Reclast). Bisphosphonates in pill form may not be recommended if you have difficulty swallowing.

• Corticosteroid-sparing agents. Your doctor may recommend other medications, either to decrease side effects or if your condition doesn't respond to corticosteroids. These medications include azathioprine (Imuran) or methotrexate (Rheumatrex). Your doctor may prescribe these alone or in combination with corticosteroids.

  When in combination, these additional immunosuppressants can be used to lessen the dose and potential side effects of the corticosteroid. Immunosuppressants, such as cyclophosphamide (Cytoxan) and cyclosporine (Neoral, Sandimmune), may improve signs and symptoms of polymyositis and interstitial lung disease.

Antibody therapy

• Intravenous immunoglobulin (IVIg). Immunoglobulin contains healthy antibodies from blood donors. High doses can block the damaging antibodies that attack muscle in polymyositis.

Immunosuppressive therapies
In addition to corticosteroids and immunosuppressive drugs, other treatments to suppress your immune system include:

• Tacrolimus (Prograf). This transplant-rejection drug may work to inhibit the immune system. Tacrolimus is often used topically to treat dermatomyositis and other skin problems. When taken orally, it may be helpful in treating people who have polymyositis complicated by interstitial lung disease.

Investigational treatment

• Biological therapies. Rituximab (Rituxan) has been studied in small numbers of people with polymyositis and dermatomyositis and shown to improve muscle strength, lung involvement and skin rash. Tumor necrosis factor (TNF) inhibitors such as etanercept (Enbrel) and infliximab (Remicade) have not been shown to be effective in trials with small numbers of people with polymyositis or dermatomyositis. Rituximab is not approved by the Food and Drug Administration for the treatment of polymyositis, so your insurance company will likely require preapproval if you wish to be reimbursed.

Other treatment

• Physical therapy. A physical therapist can show you exercises to maintain and improve your strength and flexibility and advise an appropriate level of activity.
• Dietetic assessment. Later in polymyositis, chewing and swallowing can become more difficult. A registered dietitian can teach you how to prepare easy-to-eat foods.
• Speech therapy. If your swallowing muscles are weakened by polymyositis, speech therapy can help you learn how to compensate for those changes.

Psoriatic arthritis

Definition

Psoriatic arthritis is an immune-mediated (autoimmune), connective tissue disease that is associated with a skin disorder marked by bumps and scaling (psoriasis) and is characterized by inflammation of the ligaments, tendons, fascia, and joint capsules (enthesitis) of the upper extremities, especially the hands. Small joints of the feet and large joints of the legs such as hips, knees, and ankles may be also involved. Most commonly, psoriatic arthritis affects fewer than five joints. Its arthroscopic symptoms and clinical findings are similar to those of rheumatoid arthritis, but it differs in its high frequency of distal joint involvement (joints farthest from the center of the body, such as small joints of hands and feet) and the serum of affected individuals is negative for rheumatoid factor.

Psoriatic arthritis occurs in five general patterns: arthritis affecting the small distal joints of toes and fingers (distal interphalangeal arthropathy), asymmetrical oligoarticular arthritis of the extremities, symmetrical polyarthritis that resembles rheumatoid arthritis, deforming and destructive arthritis (arthritis mutilans) with resorption of bone (osteolysis) and dissolution of the joint, and arthritis of the spine and sacroiliac joints (psoriatic spondylitis). These patterns may change in an individual over time.

The etiology of psoriatic arthritis is unknown, but genetic, immunologic and less conclusively, environmental factors such as infection and trauma are considered important components. The disease is associated with increased frequency of certain human leukocyte antigens (including HLA-B27, HLA-DR4m -DR7, and –Cw6) in the tissue of those affected with psoriasis and/or psoriatic arthritis. Genome scans have shown linkages to specific gene loci. Serum levels of immunoglobulins (IgA and IgG) are higher in psoriatic arthritis patients. Psoriasis is present years before the onset of arthritis in about 70% of individuals with psoriatic arthritis; 10% to 15% develop psoriasis and arthritis simultaneously, and 15% to 20% have arthritis before skin involvement (Ruderman). Risk: Individuals with psoriasis are at greatest risk for developing psoriatic arthritis; approximately 5% to 8% of individuals with psoriasis develop psoriatic arthritis (Hammadi). In contrast to rheumatoid arthritis, which affects women more than men, psoriatic arthritis shows no gender preference. Psoriatic arthritis usually occurs in individuals between ages 35 and 55 years, but can develop at any age. Risk is greater among whites than other races. A family history of psoriatic arthritis is a risk factor for developing the disease. Incidence and Prevalence: Incidence of psoriatic arthritis in the general population is estimated to be 1%; about 1 million adults in the US are affected (Hammadi) compared to 2 million with rheumatoid arthritis (Hammadi). Although incidence varies among countries, the incidence of psoriatic arthritis internationally ranges from 1% to 40% depending on the clinical criteria applied and whether all races are included (Van Voorhees). Prevalence is higher among whites than among African Americans and Native Americans; the disease occurs in 2.5% of white North Americans and 0.05% to 0.24% in the international white population (Hammadi).

Source: Medical Disability Advisor

Diagnosis

History: The individual may describe a family history of psoriasis or psoriatic arthritis. The individual may complain of pain, stiffness, and swelling of the joints, especially the small joints of the hands and feet. Depending on the area affected by psoriatic arthritis, other complaints may include back pain, chest pain, pain on walking or climbing stairs, pain around the eyes, and shortness of breath. Humidity and temperature changes may affect arthritis symptoms. Fever may be present in patients with the arthritis mutilans subtype of psoriatic arthritis. Individual may report that psoriasis has been present for some time before arthritis symptoms or that psoriasis developed at the same time as arthritis symptoms. The physician will obtain a history of recent and prior illness, particularly bacterial or viral infections. Physical exam: Psoriasis is usually present but may be limited to patches (lesions) between the buttocks, behind the ears, or other inconspicuous areas such as the umbilicus or scalp; psoriasis can be severe in some individuals. Swelling may be seen as well as deformity of the joints, especially finger joints in severe, chronic cases. Overgrowth of bone (hyperostosis) may be seen. "Sausage digits" (dactylitis) of the hands and feet are common in individuals with psoriatic arthritis. Affected joints often have a purplish discoloration. An accumulation of fluid may be seen in a joint (effusion). Nails of the affected digits may be pitted or crumbling (onycholysis). Inflammation of the outer membrane of the eyes (conjunctivitis) or the uveal tract (uveitis) may occur in up to 30% of individuals with psoriatic arthritis (Van Voorhees). Tests: There is no specific diagnostic test for psoriatic arthritis; observing the pattern of joint involvement is essential to correct diagnosis. Blood tests that may be helpful in making the diagnosis include erythrocyte sedimentation rate and C-reactive protein to evaluate inflammation, hemoglobin, rheumatoid factor (to rule out rheumatoid arthritis), uric acid (to rule out gout), human leukocyte antigen (HLA) typing, and antinuclear antibodies. A sample of joint fluid may be analyzed to determine the number and type of white blood cells. A cytokine profile may be done to assess T cell and monocyte activation, which may help differentiate between psoriatic arthritis and rheumatoid arthritis. X-rays usually are done to help differentiate psoriatic arthritis from other types of arthritis based on degree of joint erosion. Depending on areas affected and symptoms reported by the individual, either CT or MRI imaging can be done to examine soft tissue and joint characteristics in more detail. CT scans of the sacroiliac joint are considered to be sensitive for viewing spondylitis or sacroiliitis. MRI is especially sensitive for assessing pathology of the hands and feet.

Treatment

Treatment of the polyarthritis component focuses on controlling inflammation. It is subdivided into nonpharmacological, pharmacological, and surgical therapies. Nonpharmacological therapies include rest, wearing of splints over the affected joints, joint protection, and physical therapy. Pharmacological measures include nonsteroidal/anti-inflammatory drugs (NSAIDs), occasional corticosteroid injections into the joints, disease modifying antirheumatic drugs (DMARDs), and newer, injectable biologic agents that target tissue necrosis factor (TNF) and other cytokines. Surgery (hip or knee replacement) may be indicated in severe cases.

The psoriasis component is treated with topical therapy, systemic medications, and photochemotherapy (psoralen plus UV light). Treatment is individualized and depends on the size and location of psoriasis plaques. Immunosuppressive drugs (e.g., methotrexate) and retinoic-acid derivatives may be given in cases of severe skin involvement. These drugs have demonstrated effectiveness for both skin and joint symptoms. Cyclosporine, an antibiotic, has also demonstrated good results for psoriasis and psoriatic arthritis although it has toxic effects in some individuals.

Exercise is an important component of a treatment plan for psoriatic arthritis, resulting in reduced pain, swelling, and stiffness. Sufficient rest is also required.

Source: Medical Disability Advisor

Prognosis

Many individuals with psoriatic arthritis have mild disease with episodic flares and remissions. Because psoriatic arthritis is a chronic disease, joint deformities and movement limitations may increase over time. However, most individuals can maintain reasonable function of the affected joints. Medication can lead to periods of symptom remission. Individuals with many affected joints (polyarthritis), a family history of arthritis, early onset (younger than 20 years of age), or severe skin psoriasis have a poorer prognosis. Inflammatory arthritis of the spine (spondylitis) may develop in some individuals. Some will progress to a severe stage characterized by joint disintegration (lysis) or immobility (ankylosis).

gout

What is gout?

Gout is a kind of arthritis. It can cause an attack of sudden burning pain, stiffness, and swelling in a joint, usually a big toe. These attacks can happen over and over unless gout is treated. Over time, they can harm your joints, tendons, and other tissues. Gout is most common in men.

What causes gout?

Gout is caused by too much uric acid in the blood. Most of the time, having too much uric acid is not harmful. Many people with high levels in their blood never get gout. But when uric acid levels in the blood are too high, the uric acid may form hard crystals in your joints.

Your chances of getting gout are higher if you are overweight, drink too much alcohol, or eat too much meat and fish that are high in chemicals called purines. Some medicines, such as water pills (diuretics), can also bring on gout.

What are the symptoms?

The most common sign of gout is a nighttime attack of swelling, tenderness, redness, and sharp pain in your big toe . You can also get gout attacks in your foot, ankle, or knees. The attacks can last a few days or many weeks before the pain goes away. Another attack may not happen for months or years.

See your doctor even if your pain from gout is gone. The buildup of uric acid that led to your gout attack can still harm your joints.

How is gout diagnosed?

Your doctor will ask questions about your symptoms and do a physical exam. Your doctor may also take a sample of fluid from your joint to look for uric acid crystals. This is the best way to test for gout. Your doctor may also do a blood test to

measure the amount of uric acid in your blood.

How is it treated?

To stop a gout attack, your doctor can give you a shot of corticosteroids, or prescribe a large daily dose of one or more medicines. The doses will get smaller as your symptoms go away. Relief from a gout attack often begins within 24 hours if you start treatment right away.

To ease the pain during a gout attack, rest the joint that hurts. Taking ibuprofen or another anti-inflammatory medicine can also help you feel better. But don't take aspirin. It can make gout worse by raising the uric acid level in the blood.

To prevent future attacks, your doctor can prescribe a medicine to reduce uric acid buildup in your blood. If your doctor prescribes medicine to lower your uric acid levels, be sure to take it as directed. Most people continue to take this medicine for the rest of their lives.

Paying attention to what you eat may help you manage your gout. Eat moderate amounts of a healthy mix of foods to control your weight and get the nutrients you need. Avoid regular daily intake of meat, seafood, and alcohol (especially beer). Drink plenty of water and other fluids.

Reiter’s syndrome

Reiter’s syndrome: Introduction

Reiter's syndrome is a rare type of arthritis that causes inflammation of the urinary tract, eyes, skin, mucus membranes, and joints. Reiter's syndrome, also called reactive arthritis, is believed to occur as a reaction to certain infections of the reproductive system and the digestive system.

Infections that can lead to the complication of Reiter's syndrome include a common sexually transmitted disease called chlamydia. This is the most common cause of Reiter's syndrome. A less common cause of Reiter's syndrome is food poisoning due to Salmonella, Shigella, Yersinia or Campylobacter infection. Why some people develop Reiter's syndrome in reaction to these infections and other people don't is not known, but having a certain genetic factor called HLA-B27 increases a person's chance of developing Reiter's syndrome.

Hallmark symptoms of Reiter's syndrome affect the urinary tract, eyes, skin, mucus membranes, and joints. Complications include the development of chronic arthritis. For details about additional important complications and symptoms, refer to symptoms of Reiter's syndrome.

Making a diagnosis of Reiter's syndrome begins with taking a thorough medical history, including symptoms and history of Chlamydia infection or food poisoning, and completing a physical examination. A referral is generally made to a rheumatologist for definitive diagnosis and treatment. There is no specific test that can diagnose Reiter's syndrome. Diagnosis is made by evaluating the symptoms and interpreting them in conjunction with tests that rule out other diseases and conditions and/or increase the suspicion of a diagnosis of Reiter's syndrome.

For example, a blood rheumatoid factor (RF) test will generally be positive in rheumatoid arthritis, which has some similar symptoms, but generally negative in Reiter's syndrome. Other tests may include a C-reactive protein or erythrocyte sedimentation rate, which indicate an inflammatory process occurring somewhere in the body. A chlamydia test can diagnose the presence of a chlamydia infection, one of the infections that can lead to Reiter's syndrome. A test may also be run to check for the genetic factor HLA-B27, which increases the risk of developing Reiter's syndrome. X-rays may show some changes that are characteristic of Reiter's syndrome and may rule-out some other possible causes of symptoms.

It is possible that a diagnosis of Reiter's syndrome can be missed or delayed because symptoms can vary amongst individuals and can come and go. In addition, some symptoms may be similar to symptoms of other diseases and conditions. For more information on diseases and conditions that can mimic Reiter's syndrome, refer to misdiagnosis of Reiter's syndrome.

Treatment for Reiter's syndrome varies depending on the underlying infection, the severity of symptoms, the presence of complications, a person's age and medical history, and other factors. Reiter's syndrome cannot be cured, but treatment can help to reduce symptoms until the disorder resolves spontaneously on its own. Most people with Reiter's syndrome have a good long-term prognosis and symptoms disappear within about a year.

Treatment:

Treatment of Reiter's syndrome varies depending on the type of symptoms, the severity, and other factors. Treatment includes a multifaceted plan that addresses the symptoms and treats any underlying infection, such as chlamydia.

Reiter's syndrome cannot be cured, but treatment can minimize symptoms until the disorder resolves spontaneously on its own. Most people with Reiter's syndrome have a good long-term prognosis and symptoms disappear within about a year.

Commonly used medications for treatment of the pain and inflammation of Reiter's syndrome include acetaminophen and non-steroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen (Advil) and aspirin. These medications can have serious side effects and should only be taken as recommended by a physician. Corticosteroids may also be used to reduce inflammation. If bacterial conjunctivitis occurs, it is treated with antibiotic eye drops.

Physical therapy is also often recommended for Reiter's syndrome. Physical therapy includes exercises that can help to strengthen joints, relieve pain, and maintain flexibility and mobility.

septic arthritis

What is septic arthritis?

Septic, or infectious, arthritis is infection of one or more joints by microorganisms. Normally, the joint is lubricated with a small amount of fluid that is referred to as synovial fluid or joint fluid. The normal joint fluid is sterile and, if removed and cultured in the laboratory, no microbes will be found. With septic arthritis, microbes are identifiable in an affected joint fluid.

Most commonly, septic arthritis affects a single joint, but occasionally more joints are involved. The joints affected vary somewhat depending on the microbe causing the infection and the predisposing risk factors of the person affected. Septic arthritis is also called infectious arthritis.

What microbes cause septic arthritis?

Septic arthritis can be caused by bacteria, viruses, and fungi. The most common causes of septic arthritis are bacteria, including Staphylococcus aureus and Haemophilus influenzae. In certain "high-risk" individuals, other bacteria may cause septic arthritis, such as E. coli and Pseudomonas spp. in intravenous drug abusers and the elderly, Neisseria gonorrhoeae in sexually active young adults, and Salmonella spp. in young children or in people with sickle cell disease. Other bacteria that can cause septic arthritis include Mycobacterium tuberculosis and the spirochete bacterium that causes Lyme disease.

Viruses that can cause septic arthritis include hepatitis A, B, and C, parvovirus B19, herpes viruses, HIV (AIDS virus), HTLV-1, adenovirus, coxsackie viruses, mumps, and ebola. Fungi that can cause septic arthritis include histoplasma, coccidiomyces, and blastomyces.

Who's at Risk for Septic Arthritis?

Young children and elderly adults are most likely to develop septic arthritis. People with open wounds are also at a higher risk for septic arthritis. In addition, people with a weakened immune system and those with pre-existing conditions such as cancer, diabetes, intravenous drug abuse, and rheumatic and immune deficiency disorders have a higher risk of septic arthritis.

What Are the Symptoms of Septic Arthritis?

Symptoms of septic arthritis usually come on rapidly with intense pain, joint swelling, and fever. Septic arthritis symptoms may include:

• chills
• fatigue and generalized weakness
• fever
• inability to move the limb with the infected joint
• severe pain in the affected joint, especially with movement
• swelling (increased fluid within the joint)
• warmth (the joint is red and warm to touch because of increased blood flow)

How Is Septic Arthritis Diagnosed?

A procedure called arthrocentesis is commonly used to make an accurate diagnosis of septic arthritis. This procedure involves a surgical puncture of the joint to draw a sample of the joint fluid, known as synovial fluid. Normally, this fluid is sterile and acts as a lubricant.

In arthrocentesis, a needle is inserted into the affected joint. Fluid from the joint is collected in the needle and sent to a lab for evaluation. The lab compares the white blood cell count with normal synovial fluid, and watches the fluid for any bacterial growth. This will help the doctor determine if an infection is present, and which organism is causing it.

X-rays, MRIs, and blood tests can also be used to monitor inflammation. MRI scanning is sensitive in evaluating joint destruction. Blood tests can also be taken to detect and monitor inflammation.

What's the Treatment for Septic Arthritis?

Septic arthritis treatments include using a combination of powerful antibiotics as well as draining the infected synovial fluid from the joint. It's likely that antibiotics will be administered immediately to avoid the spread of the infection. Intravenous (IV) antibiotics may be given, which requires admission to the hospital.

Initially, empiric antibiotics are chosen to cover a wide range of infections. If the bacteria can be identified, antibiotics specific to that organism are used. It may take four to six weeks of treatment with antibiotics to ensure complete eradication of the infectious agents.

Is the Infected Fluid Drained?

Yes. Drainage of the infected area is critical for rapid clearing of the infection. Drainage is performed by removing the fluid with a needle and syringe. Often the draining occurs daily or with multiple surgical procedures. The exact method depends on the location of the joint. Warm compresses on the joint, elevation of the limb, and bed rest may be necessary.

Using arthroscopy, your doctor can irrigate the joint and remove the infected tissue. If drainage cannot be accomplished with joint aspirations or arthroscopy, open joint surgery is often necessary to drain the joint. If the fluid buildup is significant, the drains are left in place to remove excess fluid that may build up after the surgery.

juvenile idiopathic arthritis

Arthritis is a condition that affects the joints. It means one or more of your joints are swollen, painful and stiff (inflamed). Juvenile means the arthritis affects children under 16 years. Idiopathic means the cause is unknown. JIA affects about one in 1,000 children in the UK.

JIA is a chronic condition. A chronic illness is one that lasts a long time, sometimes for the rest of the affected person's life. When describing an illness, the term chronic refers to how long a person has it, not to how serious a condition is.

Types of juvenile idiopathic arthritis

There are three main types of JIA.

• Oligoarticular (or pauciarticular) JIA - the most common type, affecting only a few joints, usually the knees, ankles, elbows or wrists.
• Polyarticular JIA - the second most common type, affecting many joints including fingers and toes.
• Systemic JIA (Stills disease) - the rarest type where joint pain is part of a more general illness.

Symptoms of juvenile idiopathic arthritis

JIA affects all children differently. Symptoms may come and go over time with periods when they flare up and are worse. There may be times when your child has no symptoms at all - this is called remission.

The main symptoms of JIA are swollen, painful and stiff joints. The joint areas may look red and feel hot when you touch them. The exact symptoms and number of joints affected will vary depending on which type of JIA your child has.

Children with polyarticular JIA will have at least five or more joints affected including fingers and toes. Swelling and pain may also occur in hips, neck and jaw. Your child may also have other symptoms such as:

• nodules on their elbows
• a rash
• a fever

As well as joint pain, children with systemic JIA may have:

• a fever
• a rash
• swollen glands
• tiredness and lack of energy
• weight loss

Complications of juvenile idiopathic arthritis

JIA may affect your child's general growth. It's possible that your child's affected limbs may develop at different rates. For example if the arthritis is particularly bad in one knee the affected leg may be slightly shorter.

Anaemia may also be a problem. Anaemia is when there are too few red blood cells or not enough haemoglobin in the blood.

There is also a risk that your child may develop inflammation of the eyes (uveitis). Make sure your child has regular eye checks with an ophthalmologist (a doctor who specialises in eye health), even if he or she doesn't have any obvious symptoms.

There is a risk that systemic JIA may affect internal organs such as the tissue that covers the heart, liver or spleen. Children with systemic JIA may need to have regular check-ups.

Causes of juvenile idiopathic arthritis

The cause of JIA isn't fully understood at present but it's thought to be an autoimmune disease. An autoimmune disease is a condition caused by antibodies from the immune system attacking the body. It's possible that the tendency to develop the condition is inherited. However, it's thought that other factors are likely to be involved which are responsible for setting off this reaction of your child's immune system.

Diagnosis of juvenile idiopathic arthritis

Your GP will ask about your child's symptoms and examine him or her. Your GP may also ask you about your child's medical history.

There is no single test that can diagnose JIA and your GP will want to rule out other conditions that may be causing your child's symptoms. Your GP will usually refer your child to a paediatrician (a doctor who specialises in children's health).

Your child may need to have several tests, such as:

• blood tests to check haemoglobin levels and autoantibodies.
• X-rays, MRI, CT or ultrasound scan to check for any signs of inflammation in the joints or fluid build-up around the heart or lungs

Treatment of juvenile idiopathic arthritis

There isn't a complete cure for JIA, but there are treatments available to help control or ease the symptoms.

Your child will receive treatment from a team of health professionals. He or she will need regular check-ups to monitor his or her condition.

Self-help

Regular exercise such as swimming, running or aerobics may be helpful.

Your child will be given exercises by a physiotherapist to do at home. These will aim to reduce the pain and stiffness in your child's joints. Your child will need to do these exercises every day, even though he or she may not feel like it.

Using heat treatments, such as a hot water bottle wrapped in a towel may help to ease painful and swollen joints. A cold compress, such as ice or a bag of frozen peas, wrapped in a towel may also help. Never apply ice directly to your skin as it can give you an 'ice burn' - always place a cloth between the ice and skin.

Medicines

There are many different medicines available to help control symptoms, slow down or even stop the progression of JIA.

• Non-steroidal anti-inflammatory drugs (NSAIDs) - such as ibuprofen, can help ease pain, stiffness and swelling. For some children with mild JIA, this may be the only medicine they need to take.
• Painkillers - such as paracetamol can help ease joint pain.
• Steroids - such as prednisolone can help ease swelling. Steriods may be given as tablets, injection into a tissue or joint or through a drip into a vein.
• Disease-modifying antirheumatic drugs (DMARDs) - such as methotrexate can ease swelling and slow down the disease process. Most importantly, by controlling arthritis they can help reduce how much steroids your child needs to keep well. It may take up to three months for these medicines to have an effect.
• Etanercept is a new type of medicine and it works by blocking the action of a part of the immune system. Your child may be offered this medicine if other medicines haven't helped.

Raynaud's phenomenon

What is Raynaud's phenomenon?

Raynaud's phenomenon (RP) is a condition resulting in a particular series of discolorations of the fingers and/or the toes after exposure to changes in temperature (cold or hot) or emotional events. Skin discoloration occurs because an abnormal spasm of the blood vessels causes a diminished blood supply to the local tissues. Initially, the digit(s) involved turn white because of the diminished blood supply. The digit(s) then turn blue because of prolonged lack of oxygen. Finally, the blood vessels reopen, causing a local "flushing" phenomenon, which turns the digit(s) red. This three-phase color sequence (white to blue to red), most often upon exposure to cold temperature, is characteristic of RP.

What causes Raynaud's phenomenon?

The causes of primary and secondary RP are unknown. Both abnormal nerve control of the blood-vessel diameter and nerve sensitivity to cold exposure have been suspected as being contributing factors. The characteristic color changes of the digits are in part related to initial blood-vessel narrowing due to spasm of the tiny muscles in the wall of the vessels, followed by sudden opening (dilation), as described above. The small arteries of the digits can have microscopic thickness of their inner lining, which also leads to abnormal narrowing of the blood

What conditions have been associated with Raynaud's phenomenon?

Raynaud's phenomenon has been seen with a number of conditions, including rheumatic diseases (scleroderma, rheumatoid arthritis, systemic lupus erythematosus), hormone imbalance (hypothyroidism and carcinoid), trauma (frostbite, vibrating tools), medications (propranolol [Inderal], estrogens without additional progesterone, bleomycin [Bleoxane] used in cancer treatment, and ergotamine used for headaches), nicotine, and even rarely with cancers.

A typical Raynaud’s phenomenon attack may go something like this: You are fixing dinner and go into the freezer to get a bag of peas. They are buried in the back and you have to move things around for a moment before you find them. By the time you close the freezer door, your fingers turn white and they feel cold, numb and start to hurt. The fingers then turn blue. After 10 minutes or so they turn red, tingle, throb and get warm again.

So, the symptoms of Raynaud’s phenomenon are:

• Extreme sensitivity to cold

• Body reacts to emotional stress as if it were reacting to cold

• Skin color changes: Fingers and/or toes (and sometimes ears, lips, nose) turn white due to lack of blood flow (called pallor). The blood that’s left in the tissues loses its oxygen and the fingers turn blue (called cyanosis). Finally, the skin will turn red (called rubor) as fresh oxygenated blood returns to the fingers once the vessels open.

• Coldness, pain and numbness: A lack of oxygenated blood in the fingers triggers feelings of coldness, pain and numbness – the sensation that the hands fingers have fallen asleep.

• Warmth, tingling and throbbing: The quick return of blood to the fingers triggers feelings of warmth, tingling and throbbing, like when your hands “wake up” again.

• Skin ulcers: If your Raynaud’s phenomenon is severe and your attacks tend to last a long time, you may get painful, slow-healing sores on the tips of your fingers.

• Gangrene: In rare cases, a long-term lack of oxygen to the tissues can result in gangrene and amputation of the affected digit.

Diagnosis:

Your primary care doctor can usually determine if you have Raynaud’s phenomenon simply by listening to you describe an attack. You may even have an attack while at the doctor’s office. Determining whether the disorder is primary or secondary to an underlying disease may take some time and testing, however.

To determine whether your Raynaud’s is primary or secondary, your doctor may

• Look at the nailfold capillaries. To do this, the doctor may place a drop of oil on your skin at the base of your fingernail; you will then hold your finger under a microscope. If the capillaries are enlarged or deformed, you may have a connective tissue disease.

• Send your blood to the lab. If you have antinuclear antibodies in your blood, you may have a connective tissue disease or other autoimmune disorder. If you have an elevated erythrocyte sedimentation rate, you may have an inflammatory disorder.

• Perform tests of the blood vessels to see if there is blockage of blood flow in the arms or legs. This is often done with ultrasound, or sometimes with x-rays and dye (angiogram).

Criteria for the diagnosis of Raynaud’s phenomenon:

• Primary Raynaud’s Phenomenon
  • Periodic vasospastic attacks of pallor or cyanosis (some doctors add that the attacks should have been present for at least two years)
  • Normal nailfold capillary pattern
  • Negative antinuclear antibody test
  • Normal erythrocyte sedimentation rate
  • Absence of pitting scars or ulcers of the skin, or gangrene (tissue death) in the fingers or toes

• Secondary Raynaud’s Phenomenon
  • Periodic vasospastic attacks of pallor and cyanosis
  • Abnormal nailfold capillary pattern
  • Positive antinuclear antibody test
  • Abnormal erythrocyte sedimentation rate
  • Presence of pitting scars or ulcers of the skin, or gangrene in the fingers or toes

Treatment option:

For most people with Raynaud’s phenomenon, a conservative approach not using medicines is sufficient to control attacks. For people with more severe attacks, medications can be added.

To shorten the length of an attack once it has started, try these tips:

• Warm your hands or feet in warm (not hot) water.
• Swing your arms in large circles to increase circulation.

• Use relaxation techniques, such as deep breathing or meditation.

• Practice biofeedback methods.

Self-help treatments for preventing attacks include:

• Dress warmly: It is important not only to keep your hands and feet warm, but you also must keep your whole body warm.
  • Wear layers of loose-fitting clothing, warm socks, hats, scarves and gloves or mittens in cold weather. Mittens are warmer than gloves because they trap more air and let the fingers warm each other.
  • Keep a sweater or jacket with you at all times, even in the summer. You may need it in cold, air-conditioned buildings.

• Control your body temperature at home.

• Use flannel sheets or layers of blankets. Use an electric blanket to warm the sheets before you get into bed (make sure you follow the user instructions carefully). Consider wearing mittens and socks to bed if your hands and feet get cold when you sleep.
• Keep the rooms you use most often at a comfortable temperature.
• Start running your bath or shower water before you are ready to bathe so you don’t touch cold water. Keep the bathroom door closed so the steam will warm the room.
• Avoid cold items. Use insulated drinking glasses for your cold drinks; wear gloves when reaching in the freezer; use tepid water to rinse vegetables or hand-wash clothing.
• Use chemical warmers. Small heating pouches can be placed in your pockets, mittens or boots when you need to be outside in the cold for a while.

• Protect your skin: Poor blood flow may make your skin dry. It also may cause cuts, cracks or sores to heal more slowly than usual.
  • Use lotion with lanolin every day on your hands and feet to keep your skin from chapping or cracking.
  • Wash with a mild, creamy soap. Clean between your fingers and toes, but don’t soak them.
  • Examine your feet and hands daily to check for ulcers. If you develop one, keep it clean and covered. See your doctor right away.
  • Protect your nails. Use lotion to keep your cuticles soft. Carefully cut hangnails and file your nails in a rounded fashion to the tips of your fingers.

• Quit smoking: Chemicals in cigarette smoke constrict blood vessels – something you definitely don’t need if you have Raynaud’s phenomenon.

• Control stress: Stress and emotional upset can trigger a Raynaud’s attack, so avoiding stressful situations and learning to relax once you’re feeling anxious can lessen the number of attacks you have. Relaxation techniques and biofeedback training can be learned through a stress management program.

Medical treatments to control severe Raynaud’s phenomenon include:

• Calcium-channel blockers and alpha blockers: These drugs (such as nifedipine [Procardia] and doxazosin [Cardura]) relax smooth muscle and dilate small blood vessels. They decrease the frequency and severity of attacks and help skin ulcers heal.

• Nitroglycerine paste: This vasodilator (a drug that dilates blood vessels) can be applied to the fingers to help heal skin ulcers.

• Antibiotic ointment: In some cases needed to prevent ulcers from becoming infected.

• Analgesics: Pain-relieving drugs, sometimes containing narcotics, may be needed to control the pain of skin ulcers.

• Prostaglandin and prostacyclin: Intravenous prostaglandin and prostacyclin may be needed for some people with severely affected digits.

• Arm pump: A counterpulsation arm pump which keeps blood in the arm at higher pressures is available and may be helpful in managing ulcers of the fingers resulting from severe Raynaud’s phenomenon.

• Surgery: In severe cases, a doctor may recommend surgery to treat Raynaud’s phenomenon. Surgical procedures that are used to treat Raynaud’s phenomenon include cutting the nerves that cause narrowing of the blood vessels or performing vascular surgery to widen the blood vessels causing the Raynaud’s phenomenon. These procedures are done mainly for very severe secondary forms of the condition.

carpal tunnel syndrome

What is carpal tunnel syndrome?

Carpus is a word derived from the Greek word karpos, which means "wrist." The wrist is surrounded by a band of fibrous tissue that normally functions as a support for the joint. The tight space between this fibrous band and the wrist bone is called the carpal tunnel. The median nerve passes through the carpal tunnel to receive sensations from the thumb, index, and middle fingers of the hand. Any condition that causes swelling or a change in position of the tissue within the carpal tunnel can squeeze and irritate the median nerve. Irritation of the median nerve in this manner causes tingling and numbness of the thumb, index, and the middle fingers -- a condition known as "carpal tunnel syndrome."

Causes

Carpal tunnel syndrome occurs as a result of compression of the median nerve. The median nerve runs from your forearm through a passageway in your wrist (carpal tunnel) to your hand. It provides sensation to the palm side of your thumb and fingers, with the exception of your little finger. It also provides nerve signals to move the muscles around the base of your thumb (motor function).

In general, anything that crowds, irritates or compresses the median nerve in the carpal tunnel space can lead to carpal tunnel syndrome. For example, a wrist fracture can narrow the carpal tunnel and irritate the nerve, as can the swelling and inflammation resulting from rheumatoid arthritis.

In many cases, no single cause can be identified. It may be that a combination of risk factors contributes to the development of the condition.

Tests and diagnosis

Your doctor may conduct one or more of the following tests to determine whether you have carpal tunnel syndrome:

• History of symptoms. The pattern of your signs and symptoms may offer clues to their cause. For example, since the median nerve doesn't provide sensation to your little finger, symptoms in that finger may indicate a problem other than carpal tunnel syndrome. Another clue is the timing of the symptoms. Typical times when you might experience symptoms due to carpal tunnel syndrome include while holding a phone or a newspaper, gripping a steering wheel, or waking up during the night.
• Physical exam. Your doctor will want to test the feeling in your fingers and the strength of the muscles in your hand, because these can be affected by carpal tunnel syndrome. Pressure on the median nerve at the wrist, produced by bending the wrist, tapping on the nerve or simply pressing on the nerve, can bring on the symptoms in many people.
• X-ray. Some doctors may recommend an X-ray of the affected wrist to exclude other causes of wrist pain, such as arthritis or a fracture.
• Electromyogram. Electromyography measures the tiny electrical discharges produced in muscles. A thin-needle electrode is inserted into the muscles your doctor wants to study. An instrument records the electrical activity in your muscle at rest and as you contract the muscle. This test can help determine if muscle damage has occurred.
• Nerve conduction study. In a variation of electromyography, two electrodes are taped to your skin. A small shock is passed through the median nerve to see if electrical impulses are slowed in the carpal tunnel.

The electromyogram and nerve conduction study tests are also useful in checking for other conditions that might mimic carpal tunnel syndrome, such as a pinched nerve in your neck.

Your doctor may recommend that you see a rheumatologist, neurologist, hand surgeon or neurosurgeon if your signs or symptoms indicate other medical disorders or a need for specialized treatment.

Treatments and drugs

Some people with mild symptoms of carpal tunnel syndrome can ease their discomfort by taking more-frequent breaks to rest their hands and applying cold packs to reduce occasional swelling. If these techniques don't offer relief within a few weeks, additional treatment options include wrist splinting, medications and surgery. Splinting and other conservative treatments are more likely to help you if you've had only mild to moderate symptoms for less than 10 months.

Nonsurgical therapy
If the condition is diagnosed early, nonsurgical methods may help improve carpal tunnel syndrome. Methods may include:

• Wrist splinting. A splint that holds your wrist still while you sleep can help relieve nighttime symptoms of tingling and numbness. Nocturnal splinting may be a good option if you are pregnant and have carpal tunnel syndrome.
• Nonsteroidal anti-inflammatory drugs (NSAIDs). NSAIDs may help relieve pain from carpal tunnel syndrome in the short term. There's no evidence, though, that these drugs can actually improve the carpal tunnel syndrome itself.
• Corticosteroids. Your doctor may inject your carpal tunnel with a corticosteroid, such as cortisone, to relieve your pain. Corticosteroids decrease inflammation and swelling, which relieves pressure on the median nerve. Oral corticosteroids aren't considered as effective as corticosteroid injections for treating carpal tunnel syndrome.

If carpal tunnel syndrome results from an inflammatory arthritis, such as rheumatoid arthritis, then treating the underlying condition may reduce symptoms of carpal tunnel syndrome, but this hasn't been proved.

Surgery
If your symptoms are severe or persist after trying nonsurgical therapy, surgery may be the best option.

The goal of carpal tunnel surgery is to relieve pressure on your median nerve by cutting the ligament pressing on the nerve. During the healing process after the surgery, the ligament tissues gradually grow back together while allowing more room for the nerve than existed before. The surgery may be done a couple of different ways. Either technique has risks and benefits that are important to discuss with your surgeon before surgery.

• Endoscopic surgery. Carpal tunnel surgery can be done using an endoscope, a telescope-like device with a tiny camera attached to it that allows your doctor to see inside your carpal tunnel and perform the surgery through small incisions in your hand or wrist.
• Open surgery. In other cases, surgery involves making a larger incision in the palm of your hand over the carpal tunnel and cutting through the ligament to free the nerve.

In general, your doctor will encourage you to use your hand after surgery, gradually working back to normal use of your hand while avoiding forceful hand motions or extreme positions of your wrist. Soreness or weakness may take from several weeks to as long as a few months to resolve after surgery. If your symptoms were very severe before surgery, symptoms may not go away completely after surgery.

Rheumatic fever

Rheumatic fever is an inflammatory disease that may develop after an infection with Streptococcus bacteria (such as strep throat or scarlet fever). The disease can affect the heart, joints, skin, and brain.

Causes

Rheumatic fever is common worldwide and is responsible for many cases of damaged heart valves. It is not common in the United States, and usually occurs in isolated outbreaks. The latest outbreak was in the 1980s.

Rheumatic fever mainly affects children ages 6 -15, and occurs approximately 20 days after strep throat or scarlet fever.

Symptoms

• Abdominal pain
• Fever
• Heart (cardiac) problems, which may not have symptoms, or may result in shortness of breath and chest pain
• Joint pain, arthritis (mainly in the knees, elbows, ankles, and wrists)
• Joint swelling; redness or warmth
• Nosebleeds (epistaxis)
• Skin nodules
• Skin rash (erythema marginatum)
  • Skin eruption on the trunk and upper part of the arms or legs
  • Eruptions that look ring-shaped or snake-like
• Sydenham chorea (emotional instability, muscle weakness and quick, uncoordinated jerky movements that mainly affect the face, feet, and hands)

Exams and Tests

Because this disease has different forms, no one test can firmly diagnose it. Your doctor will perform a careful exam, which includes checking your heart sounds, skin, and joints.

Tests may include:

• Blood test for recurrent strep infection (such as an ASO test)
• Complete blood count
• Electrocardiogram
• Sedimentation rate (ESR)

Several major and minor criteria have been developed to help standardize rheumatic fever diagnosis. Meeting these criteria, as well as having evidence of a recent streptococcal infection, can help confirm that you have rheumatic fever.

The major criteria for diagnosis include:

• Arthritis in several joints (polyarthritis)
• Heart inflammation (carditis)
• Nodules under the skin (subcutaneous skin nodules)
• Rapid, jerky movements (chorea, Sydenham chorea)
• Skin rash (erythema marginatum)

The minor criteria include:

• Fever
• High ESR
• Joint pain
• Other laboratory findings

You'll likely be diagnosed with rheumatic fever if you meet two major criteria, or one major and two minor criteria, and have signs that you've had a previous strep infection.

Treatment of Rheumatic Fever

There is no actual cure for rheumatic fever but medication can be provided to alleviate the pain caused by some of the symptoms. Recurrent occurrence of the streptococcal infection can be prevented by administering penicillin to the patient. Aspirin is usually given to reduce fever and sometimes, corticosteroids are given to the patient to combat joint pain and swelling.

Administering low doses of antibiotics such as penicillin, erythromycin or sulfadiazine over a long-term period will help in preventing strep throat from occurring again.

Bed rest will be recommended for some patients in order to give the body some time to heal. Intake of fluids should be increased and the patient should have at least 6-8 glasses of water per day.

Complications of Rheumatic Fever

Depending on the severity of the initial attack of the disease, some patients may develop the following complications:

• Heart inflammation: inflammation of the muscle or lining of the heart
• Rheumatic Heart Disease: problems causes in the condition of the heart due to previous episode of rheumatic fever
• Sydenham Chorea: involuntary / spasmodic movements of the body
• Arrhythmias: heart rhythm disorder
• Endocarditis: deals with abnormalities caused to the endocardium (innermost tissues that lines the heart’s chambers) – inflammation of the heart valves or the lining of its chambers can be caused due to rheumatic fever
• Congestive heart failure: a very serious condition where the heart is unable to pump sufficient blood to the rest of the body
• Pericarditis: inflammation of the pericardium or the sac-like covering of the heart

Prevention of Rheumatic Fever

Physicians do not know how to reduce the pace of the damage caused to heart valves due to rheumatic fever. Therefore, it is best to nip this problem in the bud and promptly treat diseases caused due to streptococcal infections with the timely intake of antibiotics.

Pericarditis - constrictive

Constrictive pericarditis is long-term (chronic) inflammation of the sac-like covering of the heart (the pericardium) with thickening, scarring, and muscle tightening (contracture).

See also:

• Bacterial pericarditis
• Pericarditis
• Pericarditis after heart attack

Causes

Constrictive pericarditis is most commonly caused by conditions or events that cause inflammation to develop around the heart, including:

• Heart surgery
• Radiation therapy to the chest
• Tuberculosis

Less common causes include:

• Abnormal fluid buildup in the covering of the heart due to bacterial or viral infection or surgical complications
• Mesothelioma

Constrictive pericarditis may also develop without apparent cause.

The inflammation causes the covering of the heart to become thick and rigid, making it hard for the heart to stretch properly when it beats. As a result, the heart chambers don't fill up with enough blood. Blood backs up behind the heart, causing heart swelling and other symptoms of heart failure.

The condition is relatively rare in children.

Symptoms

Symptoms of chronic constrictive pericarditis include:

• Difficulty breathing (dyspnea) that develops slowly and gets worse
• Fatigue, excessive tiredness
• Long-term swelling (edema) of the legs and ankles
• Swollen abdomen
• Weakness

Exams and Tests

Constrictive pericarditis is very difficult to diagnose. Signs and symptoms are similar to restrictive cardiomyopathy and cardiac tamponade. Your doctor will need to rule out these conditions when making a diagnosis.

A physical exam may show that your neck veins stick out, suggesting increased blood pressure in the area. This is called Kussmaul's sign. The doctor may note weak or distant heart sounds when listening to your chest with a stethoscope.

The physical exam may also reveal liver swelling and fluid in the belly area.

The following tests may be ordered:

• Chest MRI
• Chest CT scan
• Chest x-ray
• Coronary angiography or cardiac catheterization
• ECG
• Echocardiogram

Treatment

The goal of treatment is to improve heart function. The cause must be identified and treated. This may include antibiotics, antituberculosis medications, or other treatments.

Diuretics ("water pills") are commonly prescribed in small doses to help the body remove excess fluid. Analgesics may be needed to control pain.

Decreased activity may be recommended for some patients.

A low-sodium diet may also be recommended.

The definitive treatment is a type of surgery called a pericardiectomy. This involves cutting or removing the scarring and part of the sac-like covering of the heart.

Outlook (Prognosis)

Constrictive pericarditis may be life threatening if untreated.

However, surgery to treat the condition is associated with a relatively high complication rate and is usually reserved for patients who have severe symptoms.

Possible Complications

• Cardiac tamponade
• Damage to the coronary arteries
• Heart failure
• Pulmonary edema
• Scarring of the heart muscle

When to Contact a Medical Professional

Call your health care provider if you have symptoms of constrictive pericarditis.

Prevention

Constrictive pericarditis in some cases is not preventable.

However, conditions that can lead to constrictive pericarditis should be adequately treated.

Pericardial effusion

Definition

Pericardial effusion (per-ih-KAHR-de-ul uh-FU-zhun) is the accumulation of excess fluid around the heart.

The heart is surrounded by a double-layered, sac-like structure called the pericardium. The space between the layers normally contains a very small amount of fluid.

Pericardial effusion is often related to inflammation of the pericardium that's caused by disease or injury, but pericardial effusion can also occur without inflammation. Sometimes, pericardial effusion can be caused by the accumulation of blood after a surgical procedure or injury.

When the volume of fluid exceeds the pericardium's "full" level, pericardial effusion puts pressure on the heart, causing poor heart function. If left untreated, pericardial effusion can cause heart failure or death.

What causes pericardial effusion?

Pericardial effusion, and the possible inflammation of the pericardium resulting from it (called pericarditis), can have many possible causes, including:

• Infection such as viral, bacterial or tuberculous
• Inflammatory disorders, such as lupus
• Cancer that has spread (metastasized) to the pericardium
• Kidney failure with excessive blood levels of nitrogen
• Heart surgery

Who is affected by pericardial effusions?

Since pericardial effusions are a result of many different diseases or conditions, anyone who develops one of the many conditions that can produce an effusion may be affected.

Is pericardial effusion serious?

The seriousness of the condition depends on the primary cause and size of the effusion and whether it can be treated effectively. Causes that can be treated or controlled, such as an infection due to a virus or heart failure, allows the patient to be effectively treated and remain free of pericardial effusions.

Pericardial effusion caused by other conditions, such as cancer, is very serious and should be diagnosed and treated promptly.

Additionally, rapid fluid accumulation in the pericardium can cause cardiac tamponade, a severe compression of the heart that impairs its ability to function. Cardiac tamponade resulting from pericardial effusion can be life-threatening.

What are the symptoms of pericardial effusion?

Many patients with pericardial effusion have no symptoms. The condition is often discovered on a chest x-ray or echocardiogram that was performed for another reason. Initially, the pericardium may stretch to accommodate excess fluid build-up. Therefore, signs and symptoms may not occur until a large amount of fluid has collected over time.

If symptoms do occur, they may result from compression of surrounding structures, such as the lung, stomach or phrenic nerve (a nerve that connects to the diaphragm). Symptoms also may occur due to diastolic heart failure (heart failure that occurs because the heart is unable to relax normally between each contraction due to the added compression).

Symptoms of pericardial effusion include:

• Chest pressure or pain
• Shortness of breath
• Nausea
• Abdominal fullness
• Difficulty in swallowing

Symptoms that pericardial effusion is causing cardiac tamponade include:

• Blue tinge to the lips and skin
• Shock
• Change in mental status

Cardiac tamponade is a severe compression of the heart that impairs its ability to function. Cardiac tamponade resulting from pericardial effusion can be life-threatening and is a medical emergency, requiring urgent drainage of the fluid.

How is pericardial effusion diagnosed?

The tests most commonly used to diagnose and evaluate pericardial effusion include:

• Chest x-ray
• Computed tomography (CT) scan of the chest
• Echocardiogram
• Pericardiocentesis: a procedure that uses a needle to remove fluid from the pericardium; the fluid is then examined to determine the cause of the effusion

How is pericardial effusion treated?

Treatment of pericardial effusion is based on the underlying condition that is causing it and if the effusion is leading to severe symptoms, such as shortness of breath or difficulty breathing.

Depending on the cause, the excess fluid may be either rich in protein (exudate) or watery (transudate). These two categories help physicians determine the best way to treat the cause of a pericardial effusion.

Medical management

The goal of medical management for pericardial effusions is to treat the underlying cause. Medical therapies for pericardial effusions include:

• Nonsteroidal antiinflammatory medications (NSAIDs) can be used to treat pericardial effusions caused by inflammation.
• Diuretics and other heart failure medications can be used to treat pericardial effusions caused by heart failure.
• Antibiotics are used to treat pericardial effusions caused by an infection.
• If a pericardial effusion is related to the presence of cancer, treatment may include chemotherapy, radiation therapy, or medication infused within the chest.

Procedures to treat pericardial effusion

Regardless if the pericardial effusion is transudative (consisting of watery fluid) or exudative (made up of protein-rich fluid), a large pericardial effusion causing respiratory symptoms or cardiac tamponade should be drained to remove the excess fluid, prevent its re-accumulation, or treat the underlying cause of the fluid buildup.

Large pericardial effusions may be drained through:

• Ultrasound-guided pericardiocentesis, a safe and effective procedure to remove excess fluid from the pericardium.
• Video-assisted thoracoscopic surgery (VATS), also known as thoracoscopy is a minimally-invasive technique performed under general anesthesia. VATS allows for visual evaluation of the pericardium and is used when the diagnosis of pericardial effusion has remained undiagnosed despite previous, less-invasive tests. It is also used to drain the excess fluid and prevent its reaccumulation.

Pericardial effusions that cannot be managed through medical management or drainage of excess fluid may require percutaneous (nonsurgical) or surgical treatment.

Percutaneous Balloon Pericardiotomy is a nonsurgical procedure performed using x-ray guidance to view the pericardium and place a balloon dilating catheter. Percutaneous balloon pericardiotomy is 85 to 92 percent successful at relieving reaccumulation of pericardial fluid 30 days following the procedure.

Pericardial Window (Subxyphoid Pericardiostomy) is a minimally invasive procedure in which an opening is made in the pericardium to drain fluid that has accumulated around the heart. A pericardial window can be completed through a small incision below t

Dextrocardia

Dextrocardia is a condition in which the heart is pointed toward the right side of the chest instead of normally pointing to the left. It is present at birth (congenital).

Causes

During the early weeks of pregnancy, the baby's heart develops. Sometimes, for reasons that are unclear, the heart develops and turns so that it points to the right side of the chest instead of the left side.

There are several types of dextrocardia. Most involve other defects of the heart and abdomen area.

The simplest type of dextrocardia is one in which the heart is a mirror image of the normal heart, and no other problems exist. This condition is rare. Usually in this case, the organs of the abdomen and the lungs will also be arranged in a mirror image of their normal position. For example, the liver will be on the left side instead of the right.

Some people with mirror-image dextrocardia have a problem with the fine hairs (cilia) that filter the air going into their nose and air passages. This condition is called Kartagener syndrome.

In the more common types of dextrocardia, heart defects are present in addition to the abnormal location of the heart. The most common heart defects seen with dextrocardia include:

• Double outlet right ventricle
• Endocardial cushion defect
• Pulmonary stenosis or atresia
• Single ventricle
• Transposition of the great vessels
• Ventricular septal defect

The abdominal and chest organs in babies with dextrocardia may be abnormal and may not work correctly. A very serious syndrome that appears with dextrocardia is called heterotaxy. Heterotaxy means the organs (atria of the heart and abdominal organs) are not in their usual places.

In heterotaxy, the spleen may be completely missing. Because the spleen is an extremely important part of the immune system, babies born without a spleen are in danger of severe bacterial infections and death. In another form of heterotaxy several small spleens exist, but may not work correctly.

Heterotaxy may also include:

• Abnormal gallbladder system
• Problems with the lungs
• Problems with the structure of the intestines
• Severe heart defects

Possible risk factors for dextrocardia include:

• Family history of the condition
• Mother with diabetes (may play a role in some forms of dextrocardia)

Symptoms

There are no symptoms of dextrocardia if the heart is normal.

Conditions that may include dextrocardia may cause the following symptoms:

• Bluish skin
• Difficulty breathing
• Failure to grow and gain weight
• Fatigue
• Jaundice (yellow skin and eyes)
• Pale skin (pallor)
• Repeated sinus or lung infections

Exams and Tests

There are no signs of dextrocardia if the heart is normal.

Conditions that can include dextrocardia may cause the following signs:

• Abnormal arrangement and structure of the organs in the abdomen
• Enlarged heart
• Problems with the structure of the chest and lungs, seen on x-rays
• Rapid breathing or problems breathing
• Rapid pulse

Tests to diagnose dextrocardia include:

• Computed tomography (CT) scans
• Magnetic resonance imaging (MRI) of the heart
• Ultrasound of the heart (echocardiogram)
• X-rays

Treatment

A complete mirror image dextrocardia with no heart defects requires no treatment. It is important, however, to let the child's health care provider know the heart is on the right side of the chest. This information can be important in some exams and tests.

Treatment for conditions that include dextrocardia depends on whether the infant has other heart or physical problems in addition to dextrocardia.

If heart defects are present with dextrocardia, the baby will most likely need surgery. Critically ill babies may need treatment with medication before surgery. These medications help the baby grow larger so surgery is less difficult to perform.

Medications include:

• "Water pills" (diuretics)
• Medications that help the heart muscle pump more forcefully (inotropic agents)
• Medications that lower blood pressure and ease the workload on the heart (ACE inhibitors)

The baby might also need surgery to correct problems in the organs of the abdomen.

Children with Kartagener syndrome will need repeated treatment with antibiotics for sinus infections.

Children with a missing or abnormal spleen need long-term antibiotics.

All children with heart defects should get antibiotics before surgeries or dental treatments.

See also:

• Congenital heart defect corrective surgery
• Pediatric heart surgery

Outlook (Prognosis)

Babies with simple dextrocardia have a normal life expectancy and should have no problems related to the location of the heart.

When dextrocardia appears with other defects in the heart and elsewhere in the body, how well the baby does depends on the severity of the problems.

The death rate in babies and children without a spleen may be high due to infections. This is at least partially preventable with daily antibiotics.

Possible Complications

Complications depend on whether dextrocardia is part of a larger syndrome, and whether other problems exist in the body. Complications include:

• Bacteria in the blood (septic shock)
• Blocked intestines (due to a condition called intestinal malrotation)
• Congestive heart failure
• Death
• Infection (heterotaxy with no spleen)
• Infertility in males (Kartagener syndrome)
• Repeated pneumonias
• Repeated sinus infections (Kartagener syndrome)

When to Contact a Medical Professional

Call your health care provider if your baby:

• Is often ill
• Does not seem to gain weight
• Tires easily

Seek emergency care if your baby has:

• A bluish tinge to the skin
• Trouble breathing
• Yellow skin (jaundice)

Prevention

Some syndromes that include dextrocardia may run in families. If you have a family history of heterotaxy, talk to your health care provider before becoming pregnant.

While there are no known ways to prevent dextrocardia, avoiding the use of illegal drugs (especially cocaine) before and during pregnancy may lower the risk of this problem.

Talk to your health care provider if you have diabetes, because it may contribute to your risk of having a child with certain forms of dextrocardia.

Marfan syndrome

What is Marfan syndrome?

Marfan syndrome is a heritable condition that affects the connective tissue. The primary purpose of connective tissue is to hold the body together and provide a framework for growth and development. In Marfan syndrome, the connective tissue is defective and does not act as it should. Because connective tissue is found throughout the body, Marfan syndrome can affect many body systems, including the skeleton, eyes, heart and blood vessels, nervous system, skin, and lungs.

Marfan syndrome affects men, women, and children, and has been found among people of all races and ethnic backgrounds. It is estimated that at least 1 in 5,000 people in the United States have the disorder.

What are the symptoms of Marfan syndrome?

Marfan syndrome affects different people in different ways. Some people have only mild symptoms, while others are more severely affected. In most cases, the symptoms progress as the person ages. The body systems most often affected by Marfan syndrome are:

• Skeleton - People with Marfan syndrome are typically very tall, slender, and loose-jointed. Because Marfan syndrome affects the long bones of the skeleton, a person's arms, legs, fingers, and toes may be disproportionately long in relation to the rest of the body. A person with Marfan syndrome often has a long, narrow face, and the roof of the mouth may be arched, causing the teeth to be crowded. Other skeletal problems include a sternum (breastbone) that is either protruding or indented, curvature of the spine (scoliosis), and flat feet.
  
  
• Eyes - More than half of all people with Marfan syndrome experience dislocation of one or both lenses of the eye. The lens may be slightly higher or lower than normal, and may be shifted off to one side. The dislocation may be minimal, or it may be pronounced and obvious. One serious complication that may occur with this disorder is retinal detachment. Many people with Marfan syndrome are also nearsighted (myopic), and some can develop early glaucoma (high pressure within the eye) or cataracts (the eye's lens loses its clearness).
  
  
• Heart and blood vessels (cardiovascular system) - Most people with Marfan syndrome have problems associated with the heart and blood vessels. Because of faulty connective tissue, the wall of the aorta (the large artery that carries blood from the heart to the rest of the body) may be weakened and stretch, a process called aortic dilatation. Aortic dilatation increases the risk that the aorta will tear (aortic dissection) or rupture, causing serious heart problems or sometimes sudden death. Sometimes, defects in heart valves can also cause problems. In some cases, certain valves may leak, creating a "heart murmur," which a doctor can hear with a stethoscope. Small leaks may not result in any symptoms, but larger ones may cause shortness of breath, fatigue, and palpitations (a very fast or irregular heart rate).
  
  
• Nervous system - The brain and spinal cord are surrounded by fluid contained by a membrane called the dura, which is composed of connective tissue. As someone with Marfan syndrome gets older, the dura often weakens and stretches, then begins to weigh on the vertebrae in the lower spine and wear away the bone surrounding the spinal cord. This is called dural ectasia. These changes may cause only mild discomfort; or they may lead to radiated pain in the abdomen; or to pain, numbness, or weakness in the legs.
  
  
• Skin - Many people with Marfan syndrome develop stretch marks on their skin, even without any weight change. These stretch marks can occur at any age and pose no health risk. However, people with Marfan syndrome are also at increased risk for developing an abdominal or inguinal hernia, in which a bulge develops that contains part of the intestines.
  
  
• Lungs - Although connective tissue problems make the tiny air sacs within the lungs less elastic, people with Marfan syndrome generally do not experience noticeable problems with their lungs. If, however, these tiny air sacs become stretched or swollen, the risk of lung collapse may increase. Rarely, people with Marfan syndrome may have sleep-related breathing disorders such as snoring, or sleep apnea (which is characterized by brief periods when breathing stops).

What causes Marfan syndrome?

Marfan syndrome is caused by a defect, or mutation, in the gene that determines the structure of fibrillin-1, a protein that is an important part of connective tissue. A person with Marfan syndrome is born with the disorder, even though it may not be diagnosed until later in life.

The defective gene that causes Marfan syndrome can be inherited: The child of a person who has Marfan syndrome has a 50 percent chance of inheriting the disease. Sometimes a new gene defect occurs during the formation of sperm or egg cells, making it possible for two parents without the disease to have a child with the disease. But this is rare. Two unaffected parents have only a 1 in 10,000 chance of having a child with Marfan syndrome. Possibly 25 percent of cases are due to a spontaneous mutation at the time of conception.

Although everyone with Marfan syndrome has a defect in the same gene, different mutations are found in different families, and not everyone experiences the same characteristics to the same degree. In other words, the defective gene expresses itself in different ways in different people. This phenomena is known as variable expression. Scientists do not yet understand why variable expression occurs in people with Marfan syndrome.

How Marfan syndrome diagnosed?

There is no specific laboratory test, such as a blood test or skin biopsy, to diagnose Marfan syndrome. The doctor and/or geneticist (a doctor with special knowledge about inherited diseases) relies on observation and a complete medical history, including:

• information about any family members who may have the disorder or who had an early, unexplained, heart-related death
  
  
• a thorough physical examination, including an evaluation of the skeletal frame for the ratio of arm/leg size to trunk size
  
  
• an eye examination, including a "slit lamp" evaluation
  
  
• heart tests such as an echocardiogram (a test that uses ultrasound waves to examine the heart and aorta).

The doctor may diagnose Marfan syndrome if the patient has a family history of the disease, and if there are specific problems in at least two of the body systems known to be affected. For a patient with no family history of the disease, at least three body systems must be affected before a diagnosis is made. Moreover, two of the systems must show clear signs that are relatively specific for Marfan syndrome.

In some cases, a genetic analysis may be useful in making a diagnosis of Marfan syndrome, but such analyses are often time consuming and may not provide any additional helpful information. Family members of a person diagnosed with Marfan syndrome should not assume they are not affected if there is no knowledge that the disorder existed in previous generations of the family. After a clinical diagnosis of a family member, a genetic study might identify the specific mutation for which a test can be performed to determine if other family members are affected.

Recently, doctors discovered a connective tissue disorder known as Loeys-Dietz syndrome, which has several characteristics that overlap with those of Marfan syndrome. When making a diagnosis, it is important to distinguish between the two disorders: Loeys-Dietz is more likely to cause fatal aortic aneurysms, and treatment for the two is different. A diagnostic test for Loeys-Dietz syndrome is available.

Treatment

Vision problems should be treated when possible.

Take care to monitor for scoliosis, especially during adolescence.

Medicine to slow the heart rate may help prevent stress on the aorta. Avoid participating in competitive athletics and contact sports to avoid injuring the heart. Some people may need surgical replacement of the aortic root and valve.

People with Marfan syndrome should take antibiotics before dental procedures to prevent endocarditis. Pregnant women with Marfan syndrome must be monitored very closely because of the increased stress on the heart and aorta.