Systemic Sclerosis

(Scleroderma)

An autoimmune disease characterized by fibrosis of the subcutaneous tissue and thickening of the skin over the fingers, arms, chest, and face.

Systemic sclerosis is rare among elderly persons. The cause is unknown.

Besides the characteristic skin thickening, symptoms and signs include telangiectasis, Raynaud's phenomenon, esophageal dysmotility, pulmonary fibrosis, and, occasionally, vascular involvement of the heart and kidneys. Features of systemic sclerosis sometimes occur with clinical and serologic features of SLE, polymyositis, or rheumatoid arthritis; this overlap syndrome is called mixed connective tissue disease. Autoantibodies, especially anticentromere and anti-SCL-70, are often found.

In early stages of systemic sclerosis, treatment is largely symptomatic. For example, Raynaud's phenomenon may be prevented by wearing gloves and using calcium channel blockers. Penicillamine 250 to 500 mg/day po has been advocated, but no randomized clinical trials have shown this drug to be effective. A recent meta-analysis suggests little benefit. Also, penicillamine has significant adverse effects, which may preclude use in elderly patients. Occasionally, patients present with acute renal failure and hypertensive crisis. When such fulminant presentation occurs, it is best treated with angiotensin-converting enzyme inhibitors.