Treatment of systemic sclerosis

Article Abstract:

Systemic sclerosis is a chronic disease that can be disabling, causing fatigue, pain, deformity, decreased hand function, cold intolerance, and changes in appearance. The course and severity of the disease are highly variable, so an interested, experienced physician is helpful in planning treatment; a supportive family is also important. Many therapies, including vitamins, hormones, and surgery, have been promoted, most being abandoned after objective testing. Assessment of treatments is difficult because of the rarity and variability of the disease, and consideration of the subgroup and stage of disease is important. Most patients can be classified into subgroups during the initial examination; the most common subsets are those with widespread and those with limited skin involvement. More is known about the courses of disease in these groups, and the early stages and likely complications are described. As concepts of the mechanisms underlying systemic sclerosis have only developed in the last decade, disease-modifying drugs targeting the dysfunctional reactions have not yet been identified or developed. Anti-inflammatory drugs, including steroids, have not been very useful, probably because the inflammatory phase is of relatively short duration compared with the fibrotic stage. Therapy to suppress immune function is theoretically appropriate, and the actual effectiveness is unclear, but should nevertheless be tried in patients with rapidly progressive disease. D-Penicillamine has been effective, while colchicine does not appear to be. Medications to improve blood and blood vessel problems ranging from increased clotting and blood viscosity to vasoconstriction may be helpful, but should be studied further. Supportive therapies for symptoms of Raynaud's phenomenon, finger ulcers, and skin dryness and thickening are important. Calcium deposition and joint problems should also be treated. Other problem areas needing treatment are discussed in detail and include muscle, gastrointestinal tract, lung, heart, and kidney impairments. Reversal of kidney disease by angiotensin converting enzyme inhibitors, or treatment with dialysis or transplantation, has been a significant advance. This review suggests that many symptoms of scleroderma can be treated. (Consumer Summary produced by Reliance Medical Information, Inc.)

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Fibrosing alveolitis in the adult respiratory distress syndrome

Article Abstract:

The presence of type III procollagen in the lungs appears to be a marker for poor prognosis among patients with adult respiratory distress syndrome. Collagen is the protein that makes up connective tissue. A study found that the presence of procollagen III in the lungs of patients with adult respiratory distress syndrome was associated with a higher mortality rate even after compensation for other factors that might affect survival rates. Levels of procollagen III increased within three days of onset of the disease. This association makes sense because the abnormal formation of connective tissue in the alveoli of the lung impairs respiration.

Editorial, Physiological aspects, Acute respiratory distress syndrome, Adult respiratory distress syndrome

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Pulmonary involvement in diffuse cutaneous systemic sclerosis: broncheoalveolar fluid granulocytosis predicts progression of fibrosing alveolitis

Article Abstract:

The authors present their research among patients with both fibrosing alveolitis, a progressive lung disorder and systemic sclerosis, a thickening of skin which can lead to affect internal organs as well. Their conclusion is that such patients should be treated using immunosuppresive methods.

Statistical Data Included

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