What is Felty's syndrome

What is Felty's syndrom?

Felty's syndrome is a complication of long-standing rheumatoid arthritis. Felty's syndrome is defined by the presence of 3 conditions: rheumatoid arthritis, an enlarged spleen (splenomegaly), and an abnormally low white blood count. Felty's syndrome affects less than 1% of patients with rheumatoid arthritis.

What are the symptoms of Felty's syndrome?

Some patients with Felty's syndrome have more infections, such as pneumonia or skin infections, than the average person. This increased susceptibility to infections is attributed to the low white blood counts. Ulcers in the skin over the legs can complicate Felty's syndrome.

What causes Felty's syndrome?

The cause of Felty's syndrome is not known. White blood cells are produced in the bone marrow. There seems to be an active bone marrow function in patients with Felty's syndrome despite the low white blood counts. White cells may be stored excessively in the spleen of a patient with Felty's syndrome. This is especially true in patients with Felty's syndrome that have antibodies against the particular white blood cells usually affected (called granulocytes or neutrophils).

How is Felty's syndrome diagnosed?

There is no single test for Felty's syndrome. It is diagnosed based on the presence of the three conditions mentioned above. Most patients do have rheumatoid arthritis antibodies (rheumatoid factor) in the blood.

How is Felty's syndrome treated?

Treatment of Felty's syndrome is not always required. The underlying rheumatoid arthritis is managed in the standard fashion. Treatments used for patients with recurring infections, active arthritis, or ulcer in the legs include rheumatoid disease modifying drugs, such as gold salts, methotrexate, azathioprine, and penicillamine. Patients with severe infectious diseases may benefit by weekly injections with a stimulating factor (granulocyte stimulating factor/GSF) that acts to increase the amount of white blood cells. Surgical removal of the spleen has been performed for the same reasons, but has not been evaluated by long-term research studies.

Churg Strauss Syndrome

Churg Strauss syndrome is a type of vasculitis (blood vessel inflammation) that occurs throughout the body. The American College of Rheumatology defined criteria for a diagnosis of Churg Strauss syndrome:

• Asthma
• High numbers of eosinophils, a type of white blood cell, in the blood
• Pain, numbness, or tingling in the arms and legs (called mononeuropathy)
• Lung abnormalities
• Sinus problems such as abnormal growths (polyps)
• A tissue sample showing a blood vessel with eosinophils around it

A person with Churg Strauss syndrome may have some or all of these symptoms.

Are there other symptoms?
Besides the criteria for diagnosis listed above, the person with Churg Strauss syndrome may have:

• Lumps (nodules) or raised patches (purpura) in the skin
• Kidney, abdominal, or heart problems because of blood vessel inflammation
• Weakness and fatigue (malaise)
• Loss of appetite (anorexia) and weight loss
• Other symptoms specific to the part of the body affected in the person

Who gets it?
Usually, a person already has asthma when he or she develops Churg Strauss syndrome. What brings on the syndrome is not yet clear, but research indicates it is an autoimmune disorder, meaning the body's white blood cells, which usually fight off diseases, begin attacking healthy tissue. It affects males and females equally.

How is it treated?
Since the syndrome seems to be caused by a problem with an overactive immune system, medications are given to slow down (suppress) its activity. Prednisone is the medication usually used, but other immunosuppressive drugs such as azathioprine (Imuran), mycophenolate (CellCept), methotrexate, or cyclophosphamide (Cytoxan) may be added if needed. High doses of medication can be given intravenously in severe cases. Symptoms usually start to resolve quickly after starting medication, but treatment can last for 1 or 2 years depending upon how severe the syndrome is in the individual.

What is the future for research?
Discovering what causes Churg Strauss syndrome will help doctors find a way to control it, or even prevent it from occurring. It does not seem to occur in families, so although genetics may play a part in its development, it does not seem to be inherited. It is possible that exposure to environmental toxins may play a role, as it does in other autoimmune disorders. The syndrome may be caused by an overactive immune system that was set in motion by some kind of infection. Some or all of these factors may be involved; it will be up to research to identify the precise cause(s) of the disorder.

Kawasaki Disease

Kawasaki Disease is an illness that involves the skin, mouth, and lymph nodes, and typically affects children who are under the age of 5. The cause of Kawasaki Disease is unknown, but if the symptoms are recognized early, kids with the disease can fully recover within a few days. If it goes untreated, it can lead to serious complications that can involve the heart.

Kawasaki disease occurs in 19 out of every 100,000 kids in the United States. It is most common among children of Japanese and Korean descent, but the illness can affect all ethnic groups.

This illness can't be prevented, but you can help your child by learning the telltale symptoms and signs, which typically include a fever that lasts for at least 5 days, red eyes, a body rash and severely-chapped lips and mouth. If your child shows these symptoms it's a good idea to call the doctor.

Signs and Symptoms of Kawasaki Disease

The symptoms of Kawasaki Disease typically appear in phases.

The first phase, which can last for up to 2 weeks, usually involves a persistent fever that is higher than 104 degrees Fahrenheit (39 degrees Celsius), and lasts for at least 5 days. The other symptoms that typically develop include:

• severe redness in the eyes
• a rash on the child's stomach, chest, and genitals
• red, dry, cracked lips
• swollen tongue with a white coating and big red bumps
• sore, irritated throat
• swollen palms of the hands and soles of the feet with a purple-red color
• swollen lymph nodes

During the second phase of the illness, which usually begins within two weeks of when the fever first begins, the skin on the child's hands and feet may begin to peel in large pieces. The child may also experience joint pain, diarrhea, vomiting, or abdominal pain.

Complications of Kawasaki Disease

Doctors can manage the symptoms of Kawasaki disease if they catch it early. The symptoms typically disappear within just two days of when treatment begins. Usually, if Kawasaki disease is treated within 10 days of when the first symptoms begin, no heart problems develop.

But if the illness goes untreated (time period can vary, but likely for 10-14 days and sooner in young infants), it can lead to more serious complications that involve the child's heart. Kawasaki disease can lead to vasculitis, which is an inflammation of the blood vessels. This can be particularly dangerous because it can affect the coronary arteries, which supply blood to the heart.

In addition to the coronary arteries, the heart muscle, lining, valves, or the outer membrane that surrounds the heart can become inflamed. Arrhythmias (changes in the normal pattern of the heartbeat) or abnormal functioning of some heart valves can also occur.

Diagnosing Kawasaki Disease

There is no one test to detect Kawasaki disease, so a doctor typically diagnoses it by evaluating the child's symptoms and ruling out other conditions.

Typically, a child who is diagnosed with this illness will have a fever lasting 5 or more days and at least 4 of the following symptoms:

• redness in both eyes
• changes around the lips, tongue, or mouth
• changes in the fingers and toes, such as swelling, discoloration, or peeling
• a rash in the trunk or genital area
• a large swollen lymph node in the neck
• red, swollen palms of hands and soles of feet

If Kawasaki disease is suspected, a doctor may order tests to monitor the child's heart function, which can include an echocardiogram, and other tests of heart function. A doctor may also take blood and urine samples to rule out other conditions, such as scarlet fever, measles, Rocky Mountain Spotted Fever, Stevens-Johnson syndrome, juvenile rheumatoid arthritis, and an allergic drug reaction.

Treating Kawasaki Disease

Treatment should begin as soon as possible, ideally within 10 days of when the fever first begins. Usually, a child is treated with intravenous doses of gamma globulin (purified antibodies), an ingredient of blood that helps the child's body fight off infection. The child may also be given a high-dose of aspirin to reduce the risk of heart problems.

What is Legg-Calvé-Perthes Disease

Legg-Calvé-Perthes disease is a temporary condition in children in which the ball-shaped head of the thigh bone, referred to as the femoral head, loses its blood supply. As a result, the femoral head collapses. The body will absorb the dead bone cells and replace them with new bone cells. The new bone cells will eventually reshape the femoral head of the thigh bone. Legg-Calvé-Perthes disease causes the hip joint to become painful and stiff for a period of time.

Legg-Calvé-Perthes disease goes through four phases of changes that affect the head of the femur. The phases include:

• Phase 1 - Blood supply is absent to the femoral head and the hip joint becomes inflamed, stiff, and painful. Portions of the bone turn into dead tissue. The ball of the thigh bone becomes less round in appearance on x-rays. This phase can last from several months up to one year.
  
  
• Phase 2 - The body cleans up the dead bone cells and replaces them with new, healthier bone cells. The femoral head begins to remodel into a round shape again. The joint is still irritated and painful. This phase can last from one to three years.
  
  
• Phase 3 - The femoral head continues to model itself back into a round shape with new bone. This phase lasts for one to three years.
  
  
• Phase 4 - Normal bone cells replace the new bone cells. This last phase can last a few years to complete the healing process.

What causes Legg-Calvé-Perthes disease?

The cause of Legg-Calvé-Perthes disease is unknown. It is four times more likely to occur in boys than girls.

Legg-Calvé-Perthes disease commonly affects first-born children and is typically seen in children 4 to 8 years of age. The majority of cases affect only one hip.

What are the symptoms of Legg-Calvé-Perthes disease?

The child typically complains of pain in his/her hip that is aggravated by activity. Sometimes, they will also experience pain in their thigh or knee area. The child usually walks with a limp and reports that rest will alleviate the pain.

The symptoms of Legg-Calvé-Perthes disease may resemble other conditions or medical problems of the hip. Always consult your child's physician for a diagnosis.

How is Legg-Calvé-Perthes disease diagnosed?

In addition to a complete medical history and physical examination, diagnostic procedures for Legg-Calvé-Perthes disease may include:

• x-rays - a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.
  
  
• bone scans - a nuclear imaging method to evaluate any degenerative and/or arthritic changes in the joints; to detect bone diseases and tumors; to determine the cause of bone pain or inflammation.
  
  
• magnetic resonance imaging (MRI) - a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body.
  
  
• arthrograms - a diagnostic imaging test to study the non-bony structures of joints.
  
  
• blood tests

Treatment for Legg-Calvé-Perthes disease:

Specific treatment for Legg-Calvé-Perthes disease will be determined by your child's physician based on:

• your child's age, overall health, and medical history
• the extent of the condition
• your child's tolerance for specific medications, procedures, or therapies
• expectations for the course of the condition
• your opinion or preference

The goal of treatment is to preserve the roundness of the femoral head and to prevent deformity while the condition runs its course. Treatment options are dependent upon the amount of hip pain, stiffness, and x-ray changes over time, as well as how much of the femoral head has collapsed.

Typically, the first step of treatment is to regain hip motion and eliminate pain that results from the tight muscles around the hip and the inflammation inside the joint. Treatment may include:

• rest
  
  
• activity restrictions
  
  
• medications
  
  
• bed rest and traction
  
  
• casting or bracing (to hold the femoral head in the hip socket, permit limited joint movement, and allow the femur to remold itself into a round shape again)
  
  
• surgery (to hold the femoral head in the hip socket)
  
  
• physical therapy (to keep the hip muscles strong and to promote hip movement)
  
  
• crutches or wheelchair (in some cases)

Long-term outlook for a child with Legg-Calvé-Perthes disease:

The ultimate goal in Legg-Calvé-Perthes disease is to diagnose the condition early in order to allow as much time as possible to let the femoral head remodel back into a round shape. Other treatment goals include controlling pain, maintaining hip motion, and preventing continued hip deformity.

The two most critical factors that determine the outcome are the child's age and how much of the femoral head is affected by this condition.

The more severe the case, the greater the likelihood that the child may experience limited hip motion, differences in leg lengths, and further hip problems in adulthood.

What is Behcet's syndrome

Behcet's syndrome is classically characterized as a triad of symptoms that include recurring crops of mouth ulcers (apthous ulcers), genital ulcers, and inflammation of a specialized area around the pupil of the eye (the uvea). The inflammation of the area of the eye that is around the pupil is called uveitis. Behcet's syndrome is also sometimes referred to as Behcet's disease.

The cause of Behcet's syndrome is not known. The disease is more frequent and severe in patients from the Eastern Mediterranean and Asia than those of European descent. Both inherited (genetic) and environmental factors, such as microbe infections, are suspected to be factors that contribute to the development of Behcet's syndrome. Behcet's syndrome is not felt to be contagious.

What are symptoms of Behcet's syndrome?

The symptoms of Behcet's syndrome depend on the area of the body affected. Behcet's syndrome can involve inflammation of many areas of the body. These areas include the arteries that supply blood to the body's tissues. Behcet's syndrome can also affect the veins that take the blood back to the lungs to replenish the oxygen content. Other areas of body that can be affected by the inflammation of Behcet's syndrome include the back of the eyes (retina), brain, joints, skin, and bowels.

The mouth and genital ulcers are generally painful and recur in crops (many shallow ulcers at the same time). They range in size from a few millimeters to 20 millimeters in diameter. The mouth ulcers occur on the gums, tongue, and inner lining of the mouth. The genital ulcers occur on the scrotum and penis of males and vulva of women and can leave scars.

Inflammation of the eye, which can involve the front of the eye (uvea) causing uveitis, or the back of the eye (retina) causing retinitis, can lead to blindness. Symptoms of eye inflammation include pain, blurred vision, tearing, redness, and pain when looking at bright lights. It is very important for patients to have this sensitive area monitored by an eye specialist (ophthalmologist).

If the arteries become inflamed (arteritis) in patients with Behcet's syndrome, it can lead to death of the tissues whose oxygen supply depends on these vessels. This could cause at stroke if affecting the brain, belly pain if affecting the bowel, etc. When veins become inflamed (phlebitis), the inflammation can involve large veins that develop blood clots which can loosen to cause pulmonary embolism.

Symptoms of inflammation of the brain or tissue that covers the brain (meninges) include headaches, neck stiffness, and is often associated with fever. Inflammation of the brain (encephalitis) and/or the meninges (meningitis) can cause damage to nervous tissue and lead to weakness or impaired function of portions of the body. This can result in confusion and coma. Typically these features occur later in the disease course, years after the diagnosis.

Joint inflammation (arthritis) can lead to swelling, stiffness, warmth, pain, and tenderness of joints in patients with Behcet's syndrome. This occurs in about half of patients with Behcet's syndrome at some time during their lives. Knees, wrists, ankles, and elbows are the most common joints affected.

The skin of patients with Behcet's syndrome can develop areas of inflammation which spontaneously appear as raised, tender, reddish nodules (erythema nodosum), typically on the front of the legs. Some patients with Behcet's syndrome develop a peculiar red or blistery skin reaction in places where they have been pierced by blood-drawing needles (see pathergy test in diagnosis section below). Recent research has found that acne occurs more frequently in patients with Behcet's syndrome that also have arthritis as a manifestation.

Ulcerations can occur at any location in the stomach, large or small bowel in patients with Behcet's disease.

What are symptoms of Behcet's syndrome

The symptoms of Behcet's syndrome depend on the area of the body affected. Behcet's syndrome can involve inflammation of many areas of the body. These areas include the arteries that supply blood to the body's tissues. Behcet's syndrome can also affect the veins that take the blood back to the lungs to replenish the oxygen content. Other areas of body that can be affected by the inflammation of Behcet's syndrome include the back of the eyes (retina), brain, joints, skin, and bowels.

The mouth and genital ulcers are generally painful and recur in crops (many shallow ulcers at the same time). They range in size from a few millimeters to 20 millimeters in diameter. The mouth ulcers occur on the gums, tongue, and inner lining of the mouth. The genital ulcers occur on the scrotum and penis of males and vulva of women and can leave scars.

Inflammation of the eye, which can involve the front of the eye (uvea) causing uveitis, or the back of the eye (retina) causing retinitis, can lead to blindness. Symptoms of eye inflammation include pain, blurred vision, tearing, redness, and pain when looking at bright lights. It is very important for patients to have this sensitive area monitored by an eye specialist (ophthalmologist).

If the arteries become inflamed (arteritis) in patients with Behcet's syndrome, it can lead to death of the tissues whose oxygen supply depends on these vessels. This could cause a stroke if affecting the brain, belly pain if affecting the bowel, etc. When veins become inflamed (phlebitis), the inflammation can involve large veins that develop blood clots which can loosen to cause pulmonary embolism.

Symptoms of inflammation of the brain or tissue that covers the brain (meninges) include headaches, neck stiffness, and is often associated with fever. Inflammation of the brain (encephalitis) and/or the meninges (meningitis) can cause damage to nervous tissue and lead to weakness or impaired function of portions of the body. This can result in confusion and coma. Typically these features occur later in the disease course, years after the diagnosis.

Joint inflammation (arthritis) can lead to swelling, stiffness, warmth, pain, and tenderness of joints in patients with Behcet's syndrome. This occurs in about half of patients with Behcet's syndrome at some time during their lives. Knees, wrists, ankles, and elbows are the most common joints affected.

The skin of patients with Behcet's syndrome can develop areas of inflammation which spontaneously appear as raised, tender, reddish nodules (erythema nodosum), typically on the front of the legs. Some patients with Behcet's syndrome develop a peculiar red or blistery skin reaction in places where they have been pierced by blood-drawing needles (see pathergy test in diagnosis section below). Recent research has found that acne occurs more frequently in patients with Behcet's syndrome that also have arthritis as a manifestation.

How is Behcet's syndrome diagnosed

Behcet's syndrome is diagnosed based on the finding of recurrent mouth ulcerations combined with any two of the following: eye inflammation, genital ulcerations, or skin abnormalities mentioned above. A special skin test called a pathergy test can also suggest Behcet's syndrome. (The other criteria above are still required for ultimate diagnosis.) This test consists of pricking the skin of the forearm with a sterile needle. The test is called positive and suggests Behcet's syndrome when the puncture causes a sterile red nodule or pustule that is greater than two millimeters in diameter at 24 to 48 hours.

Tests, such as skin biopsy, lumbar puncture, MRI scan of the brain, and bowel tests are considered based upon symptoms that are present.

What is the treatment of Behcet's syndrome?

The treatment of Behcet's syndrome depends on the severity and the location of its manifestations in an individual patient.

Steroid (cortisone) gels, pastes (such as Kenolog in Orabase) and creams can be helpful for the mouth and genital ulcers. Colchicine can also minimize recurrent ulcerations. Mouth and genital ulcers healed and were reported at a national meeting of the American College of Rheumatology as less frequent in 9 or 12 patients who were treated with Trental (pentoxifylline). Trental also seemed to maintain the healed ulcers for up to the 29 months of the study. The effectiveness of Trental, the researchers said, seemed to be enhanced by the combination with colchicine in some patients.

Joint inflammation can require non-steroidal anti-inflammatory drugs (such as ibuprofen and others) or oral steroids. Colchicine and oral and injectable cortisone are used for inflammation involving the joints, eyes, skin, and brain. Sulfasalazine has been effective in some patients for arthritis.

Bowel disease is treated with oral steroids and sulfasalazine (Azulfidine).

Diligent treatment of eye inflammation is essential. Patients with eye symptoms or a history of eye inflammation should be monitored by an ophthalmologist. Recent research has reported successful management of resistant eye inflammation with new biologic medications that block a protein that plays a major role in initiating inflammation, called TNF. These TNF-blocking medications, including infliximab (Remicade) and etanercept (Enbrel), can also be helpful for severe mouth ulcerations.

Severe disease of the arteries, eyes, and brain can be difficult to treat and require powerful medications that suppress the immune system called immunosuppressive agents. Immunosuppressive agents used for severe Behcet's syndrome include chlorambucilazathioprine (Imuran), and cyclophosphamide (Cytoxan). Cyclosporine has been used for resistant disease. (Leukeran),

Recent studies suggest that thalidomide may be of benefit for certain patients with Behcet's syndrome in treating and preventing ulcerations of the mouth and genitals. Side effects of thalidomide include promoting abnormal development of fetal growth, nerve injury (neuropathy), and hypersedation.

Recent research further suggests that patients who have had heart valve replacement because of severe damage as a result of inflammation can benefit by immune suppression with azathioprine and prednisone after operation.

Trials are currently underway evaluating interferon alpha for the treatment of eye disease in patients with Behcet's syndrome.

What is the treatment of Behcet's syndrome

The treatment of Behcet's syndrome depends on the severity and the location of its manifestations in an individual patient.

Steroid (cortisone) gels, pastes (such as Kenolog in Orabase) and creams can be helpful for the mouth and genital ulcers. Colchicine can also minimize recurrent ulcerations. Mouth and genital ulcers healed and were reported at a national meeting of the American College of Rheumatology as less frequent in 9 or 12 patients who were treated with Trental (pentoxifylline). Trental also seemed to maintain the healed ulcers for up to the 29 months of the study. The effectiveness of Trental, the researchers said, seemed to be enhanced by the combination with colchicine in some patients.

Joint inflammation can require non-steroidal anti-inflammatory drugs (such as ibuprofen and others) or oral steroids. Colchicine and oral and injectable cortisone are used for inflammation involving the joints, eyes, skin, and brain. Sulfasalazine has been effective in some patients for arthritis.

Bowel disease is treated with oral steroids and sulfasalazine (Azulfidine).

Diligent treatment of eye inflammation is essential. Patients with eye symptoms or a history of eye inflammation should be monitored by an ophthalmologist. Recent research has reported successful management of resistant eye inflammation with new biologic medications that block a protein that plays a major role in initiating inflammation, called TNF. These TNF-blocking medications, including infliximab (Remicade) and etanercept (Enbrel), can also be helpful for severe mouth ulcerations.

Severe disease of the arteries, eyes, and brain can be difficult to treat and require powerful medications that suppress the immune system called immunosuppressive agents. Immunosuppressive agents used for severe Behcet's syndrome include chlorambucilazathioprine (Imuran), and cyclophosphamide (Cytoxan). Cyclosporine has been used for resistant disease. (Leukeran),

Recent studies suggest that thalidomide may be of benefit for certain patients with Behcet's syndrome in treating and preventing ulcerations of the mouth and genitals. Side effects of thalidomide include promoting abnormal development of fetal growth, nerve injury (neuropathy), and hypersedation.

Recent research further suggests that patients who have had heart valve replacement because of severe damage as a result of inflammation can benefit by immune suppression with azathioprine and prednisone after operation.

Trials are currently underway evaluating interferon alpha for the treatment of eye disease in patients with Behcet's syndrome.

Adult Onset Still's Disease

It's mysterious and it's rare. A form of rheumatoid arthritis, Still's Disease mainly affects children. But there's even a rarer form which strikes adults -- Adult Onset Still's Disease (AOSD).

AOSD is an inflammatory condition that attacks internal organs, joints and other parts of the body. It can appear and disappear suddenly. In very severe cases, AOSD becomes chronic and extremely debilitating, causing terrible pain and stiffness. After many years, the disease cripples vital organs such as the heart and lungs.

There are treatments, mainly anti-inflammatory drugs, that help with most AOSD symptoms. Until recently, we have known little about its causes but medical researchers are starting to learn more about AOSD. With this knowledge comes the promise of new and better treatments.

AOSD gets its name from Dr. Still, a renowned British pediatrician, who first identified the childhood form of the disease. Though rare, it is found worldwide and predominantly affects young adults between 16 and 35.

AOSD does not seem to run in families. The disease can be difficult to diagnose, as it is difficult to distinguish from a number of other conditions that cause unexplained fever as well as other forms of rheumatoid arthritis, as well as from a number of other diseases.

Symptoms of AOSD
Common symptoms of AOSD include those on the following list. It is important to remember that there is no definite pattern that AOSD symptoms follow. In some cases, only one or two symptoms occur immediately; others may follow later. Aside from fever and joint pain, which are almost always among the first signs of AOSD, some of the symptoms on this list may not appear for weeks, or even at all.

Fever
The first sign that someone may be coming down with AOSD is a high spiking fever of 103 degrees or above. The fever usually rises rapidly in the evening and then subsides to or near normal by the next morning. Occasionally, an unusual pattern of two fever spikes in a day is seen in one-fifth of AOSD sufferers.

Rash
The typical Still's rash is salmon pink in color and comes and goes unpredictably, although it sometimes occurs at the same time as high fever. It is most common on the chest and thigh, although it may also appear on the face, hands and feet. In some cases, the rash becomes itchy.

Joint and Muscle Pain
At some point, every AOSD sufferer experiences intense joint pain, often in the hand. This can be accompanied by severe muscle pain as well. Both may become worse during a fever spike.

Sore Throat
Most AOSD sufferers come down with a sore throat that is usually described as a severe, constant burning pain in the area around the pharynx.²

Heart and Lung Symptoms
These include pain, difficulty breathing and other problems that can be mistaken for an infection of tissue within the heart.

Abdominal Pain
This can be caused by swollen lymph nodes, as well as enlargement of the spleen or liver. These symptoms are also sometimes attributed to other causes.

Abnormal White Blood Cell Count and Other Blood Abnormalities
These can be easily detected using a variety of blood tests. Most patients with AOSD have greatly elevated white blood cell counts which may lead their doctors to suspect an underlying infection or even leukemia. However, the cultures of the blood and other sites are always negative and the blood and bone marrow do not show the abnormal cells found in leukemia. A group of tests referred to as "acute phase reactants" are almost always abnormally elevated in AOSD. The two most commonly associated with AOSD are an elevated sedimentation rate (ESR) and a high blood ferritin level.

Diagnosis of AOSD
While there is no simple test for AOSD, most doctors will make the diagnosis if a person has five or more of the criteria below, including two or more from the first (major criteria) list:⁷

Major AOSD Criteria

1. High fever lasting one week or longer
2. Joint pain lasting two weeks or longer
3. Rash
4. Abnormal white blood cell count and other blood problems

Minor AOSD Criteria

1. Sore throat
2. Swelling of the lymph nodes or spleen
3. Liver problems
4. Absence of rheumatoid arthritis

Joint and Muscle Change
Occasionally the diagnosis of AOSD may be supported by the finding of soft tissue swelling, bone loss and damage to the wrist, hand, hip, knee and spinal joints. These changes, however, are diagnostic.

Diseases that Mimic AOSD Symptoms
An important part of detecting AOSD is ruling out other diseases that can cause similar symptoms. Some of the prime suspects are:

• Infections such as hepatitis, rubella, parvovirus,coxsackie, EBV (Epstein-Barr virus) CMV (cytomegalo virus) and HIV (AIDS)
• Infective endocarditis (an infection of the heart tissue)
• Tuberculosis
• Lyme disease
• Ehrlichiosis
• Sarcoidosis
• Hepatitis
• Crohn's disease
• Cancer, including leukemia and lymphoma
• Connective tissue disease, such as SLE (Lupus)

Causes of AOSD
We do not know the exact cause AOSD but researchers are currently investigating a number of possibilities:

• Infection
  The abrupt appearance of high fever, sore throat, rash, lymph node swelling and abnormal white blood cell counts suggest some kind of infectious trigger.
  
  While no particular cause of infection has been found, the rubella (German Measles) virus has been detected in many people with AOSD cases. Researchers have also found some association between AOSD and EB, CMV, mumps, parainfluenza, and other viruses.
• Immune Disorder
• Genetics
• Hormonal Influences
  Pregnant women are slightly more likely to come down with AOSD or to have a relapse. On the other hand, if the female hormones associated with pregnancy played a role, we would expect AOSD to be more common in one sex or the other. Unlike lupus or rheumatoid arthritis, however, which are more common in women, AOSD seems to affect both sexes equally.

Treatment and Prognosis
The most widely used treatment for AOSD symptoms is non-steroidal anti-inflammatory drugs (NSAIDs). A good early indication of how well an AOSD sufferer will do in the long term is their initial response to these drugs. Statistically, 20-25% of AOSD sufferers improve with NSAIDs. Many of these find that their symptoms clear up quickly, sometimes for good. NSAIDs are normally taken until 1-3 months after the symptoms are gone.

In particularly acute or severe cases, doctors sometimes also prescribe corticosteroids to address heart, blood, and other life-threatening problems that AOSD can cause. Anti-TNF therapy, aimed at a component of the immune system, may be a promising treatment.¹⁰

Some AOSD sufferers do not respond well to NSAIDs and go on to develop the chronic (long term) form of the disease. Because corticosteroids have serious side effects, they cannot be used to treat AOSD over a period of years. Instead, depending on the individual case, doctors prescribe drugs such as methotrexate, IM gold, D-penicillamine, hydroxychloroquine and azathioprine. For safety reasons, cyclophosphamide is reserved only for the most difficult cases.

Summary
Though not common, AOSD is a serious and potentially debilitating disease that can cause serious damage and other problems to the joints, heart, lungs, liver and spleen. While some AOSD sufferers have only a mild form of the disease that responds well to safe NSAID drugs, others come down with a severe, chronic form that is more difficult to treat that can cause long term pain and disability or even death. While we have a lot to learn about how AOSD works and how to treat it, some progress is being made.

Cogan Syndrome

Cogan syndrome: Arteritis (also referred to as vasculitis) that involves the ear. This condition is called Cogan syndrome after the American ophthalmologist David Glendenning Cogan (1908-93) who first described it.

Cogan syndrome features not only problems of the hearing and balance portions of the ear, but also inflammation of the front of the eye (the cornea) and often fever, fatigue, and weight loss. Joint and muscle pains can also be present. Less frequently, the arteritis can involve blood vessels elsewhere in the body as in the skin, kidneys, nerves, and other tissues and organs.

Cogan syndrome can lead to deafness or blindness.

The treatment of Cogan syndrome is directed toward stopping the inflammation of the blood vessels. Cortisone-related medications, such as prednisone, are often used. Some patients with severe disease can require immune suppression medications, such as cyclophosphamide(CYTOXAN).

Cogan syndrome is extremely rare and its cause is not known.

Bicipital tendinitis

Bicipital tendinitis is an inflammation of one of the tendons that attach the muscle (biceps) on the front of the upper arm bone (humerus) to the shoulder joint. The inflammation usually occurs along the groove (bicipital groove) where the tendon passes over the humerus to attach just above the shoulder joint. See an illustration of the shoulderClick here to see an illustration..

Causes may include:

* Overuse, especially in athletes who use throwing motions.
* Inflammatory conditions, such as arthritis or bursitis.
* Infection.
* Injury.

Symptoms may include:

* Shoulder pain that may spread down the front of the arm.
* Tenderness where the biceps meets the shoulder (bicipital groove).
* Decreased shoulder movement and pain.
* A bulge in the biceps (if one of the biceps tendons ruptures).

Treatment includes rest, ice, compression, and elevation (RICE), gentle exercise, stopping smoking, and nonsteroidal anti-inflammatories (NSAIDs). Smoking can interfere with healing of tendinitis by decreasing blood supply and delaying tissue repair. A sling may be used temporarily for comfort. In rare cases, surgery is recommended if home treatment does not help.