Dr. Shiel Gives Perspectives Of Interest On Rare Rheumatic Conditions From 2001 Annual Scientific Meeting Of The American College Of Rheumatology
Below are perspectives on key reports presented at the recent national meeting of the American College of Rheumatology:
Rheumatic conditions include a wide variety of both common and rare diseases. We have all heard about the common diseases, such as rheumatoid arthritis. The rare diseases often do not make it into the public arena and also can have limited access to research. At our national rheumatology meeting, rheumatologists, like myself, are delighted to be able to explore the latest research on these rare conditions. Now, from MedicineNet.com you can too!
--Dr. Shiel
Polymyositis is an inflammatory disease of the muscle. The cause of this disease is not known. Polymyositis begins when white blood cells, which are the immune cells of inflammation, spontaneously invade muscles. The muscles affected are mostly those closest to the trunk or torso. This results in weakness which can be severe. It is a chronic illness with periods of increased symptoms, called flares or relapses, and decreased symptoms, known as remissions. The diagnosis of polymyositis involves a physical examination of muscle strength, blood tests for muscle enzymes, electrical tests of the muscle and nerves, and confirmation with a muscle biopsy. The treatment of polymyositis involves high doses of cortisone-related medications, immune suppression, and physical therapy.
HIV (AIDS virus) polymyositis is more mild than polymyositis without HIV.
Dr. Shiel's Perspective: The onset of polymyositis in these HIV infected patients occurred randomly, at anytime during the course of their infection (early or late). The disease seemed to respond well to cortisone therapy (steroids). This is some good news for persons who are affected by both conditions.
Oversized muscle enzymes (Macro CPKs) can be a cause of elevated blood enzyme levels.
Dr. Shiel's Perspective: This report is interesting for doctors. We check CPK blood levels when evaluating the heart, muscles, and brain because the enzyme can come from each of these areas in a characteristic form. Sometimes, rheumatologists are asked to evaluate patients with elevated CPKs who do not have any noticeable problems with their heart, muscles, or brain. This report from the national meeting explains a new test for a special form of CPK that stays elevated in the blood because it is bound by an antibody (immunoglobulin). Because the evaluation of CPKs can involve a muscle biopsy, the detection of a Macro CPK could spare a patient from unnecessary testing. The researchers told me that the test must be ordered as a "macro CPK level by gel filtration on sephacryl." I will be giving this a try in patients with elevated CPK levels who don't have muscle weakness.
Eosinophilic fasciitis is a specific disease of the skin that leads to inflammation and thickening of the skin and fascia underneath. The fascia is a lining tissue under the skin that covers a surface of underlying tissues. When the fascia is inflamed, the condition is referred to as "fasciitis." In patients with eosinophilic fasciitis, the involved fascia is inflamed with the eosinophil type of white blood cells. This leads to symptoms of progressive thickening and often redness, warmth, and hardness of the skin surface. For aggressive eosinophilic fasciitis, cortisone medications (such as prednisone and prednisolone) are considered along with the use of immune suppression medications (such cyclophosphamide/Cytoxan and penicillimine/Depen, Cuprimine).
Methotrexate was shown to provide an added benefit in treating eosinophilic fasciitis.
Dr. Shiel's Perspective: The research here strongly suggests that methotrexate could be helpful in treating eosinophilic fasciitis. Only seven patients were reported, but all seven improved. Furthermore, their requirement for cortisone-related medications (steroids) was reduced.
Remicade (infliximab) was beneficial in treating severe, adult-onset Still's Disease-reported by 2 research groups.
Dr. Shiel's Perspective: This report is encouraging to those affected by this severe disease. The intravenous infusions of Remicade helped decrease the signs and symptoms of the Still's Disease in all patients, including fever, arthritis, muscle pains, enlarged spleen, and rash.
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