Scleroderma

Scleroderma is a disease of progressive systemic fibrosis

Pathophysiology

1) progressive systemic sclerosis owing to deposition in tissues of circulating immune complexes that cause systemic manifestations 2) initiating event is unknown 3) mild form occurs in CREST syndrome (calcinosis, Raynaud’s phenomenon, esophageal dysfuction, sclerodactyly, telangiectasias

Signs and Symptoms

1) scleroderma often presents with Raynaud’s phenomenon 2) edema of fingers and hands followed by tightening and thickening of skin 3) ulcerations of fingertips 4) polyarthralgias 5) stone facies/restricted eye and mouth movements 6) dysphagia (esophageal hypomotility) 7) decreased intestinal motiliy with bacterial overgrowth/malabsorption 8) dyspnea on exertion 9) the “sclerodermal renal crisis” of progressive renal failure, microangiopathic hemolytic anemia, and acute onset of uncontrollable hypertension

Characteristic Test Findings in Scleroderma

Laboratory – 1) anticentromere antibodies 2) antinuclear antibodies 3) antibodies to SCL-70 (topoisomerase I) 4) presence of male fetal cells 5) decreased CD8 T suppressor cells 6) antibodies against thyroid, salivary glands, and smooth muscle and type I and type IV collagen 7) positive rheumatoid factor