Amyloidosis in the rheumatic diseases

Article Abstract:

Amyloidosis, a metabolic disorder, is characterized by the deposition of a waxy compound called amyloid that interferes with the function of vital body organs. The disease may result from disorders of the immune system, the body's natural defense system. Amyloidosis occurs in patients with the following diseases: rheumatoid arthritis, an inflammatory joint disease; ankylosing spondylitis, an arthritic disease affecting the spine; and psoriatic arthritis, an inflammatory joint disease associated with psoriasis, a skin disorder. In contrast, amyloidosis rarely occurs in: systemic lupus erythematosus, an inflammatory disease of the connective tissue; progressive systemic sclerosis, characterized by thickening and hardening of tissue throughout the body; and Reiter's disease, inflammation of the urethra, joints, and membrane lining the eyelids. New imaging methods have been developed to assess the prevalence of amyloidosis in these diseases. Factors that increase the risk of developing amyloidosis include unusually active disease and persistently high levels of the precursor serum amyloid A (SAA). Cytotoxic drugs, agents that kill cells, have been used to reduce levels of SAA and are used to treat amyloidosis, although there are no methods of removing amyloid deposits. A better understanding of factors causing amyloidosis will contribute to the development of methods for preventing and treating this life-threatening condition. (Consumer Summary produced by Reliance Medical Information, Inc.)

Risk factors, Drug therapy, Amyloidosis, Arthritis

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Axillary vein thrombosis in adolescent onset systemic sclerosis

Article Abstract:

Progressive systemic sclerosis is a rare condition in adults and even more uncommon in children. It is characterized by widespread fibrosis, or formation of fiber-like tissue, and may result from disorders of the small blood vessels and arteries. Impaired function of the endothelial cells lining the inside of the blood vessels and factors within the blood vessels may contribute to conditions that promote blood clot formation, although thrombosis (the development of blood clots) is not a common complication. A case is described of a 16-year-old girl with systemic sclerosis who developed axillary vein thrombosis, a blood clot in a vein situated in the armpit area. The patient had abnormalities of fibrinolysis (the process of breaking down clots) and endothelial cell antibodies (abnormal immune proteins that attack endothelial cells). Anti-endothelial antibodies have been previously detected in 74 percent of patients with systemic lupus erythematosus, an inflammatory disease of connective tissue; in 30 percent of patients with systemic sclerosis; and in 28 percent of patients with rheumatoid arthritis, an inflammatory joint disease. The patient also had increased levels of von Willebrand factor, which indicated blood vessel and connective tissue disease. The relation between systemic sclerosis and the development of deep vein thrombosis requires further investigation. (Consumer Summary produced by Reliance Medical Information, Inc.)

Case studies, Evaluation, Abnormalities, Chronic diseases, Pediatric diseases, Systemic scleroderma, Scleroderma (Disease), Thrombosis, Chronic diseases in children, Sclerosis, Axillary vein

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Does it take sugar? A clinical role for measuring the glycosylation of IgG?

Article Abstract:

Elevated levels of blood sugars lacking the component galactose (Gal(0)) may play a role in indicating the progression of rheumatoid arthritis (RA). Galactose is a sugar molecule attached to the immune protein, immunoglobulin G (IgG). Researchers have found that patients with rheumatic diseases have elevated Gal(0) blood levels while patients with viral, bacterial, or autoimmune diseases have normal Gal(0) blood levels. Other studies have shown that an elevated Gal(0) blood level, age greater than 51 years, low grip strength, and female gender were the most important predicting factors in the development of RA. Another study of 8 pregnant women with RA showed a decrease to normal Gal(0) blood levels associated with remission experienced during the last trimester of pregnancy. After birth, the Gal(0) blood levels returned to their previously elevated levels and disease symptoms returned.

Health aspects, Glycosylation, Immunoglobulin G

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