Scleroderma is an autoimmune disorder whose hallmark is increased collagen deposition and hardening of the skin.
While research continues to evolve our understanding of this disease, the main feature is abnormal function of fibroblast cells leading to the increased collagen deposition.
The increased deposition can lead to changes in the skin, kidneys, gastrointestinal tract, and other areas. This family of diseases often shares clinical features with inflammatory muscle diseases, mixed connective tissue disease (MCTD), rheumatoid arthritis, and Sjogren’s syndrome. Many different organ systems can become involved including the pulmonary and cardiac systems.
The classically described forms are diffuse cutaneous systemic sclerosis (common referred to as “scleroderma”), and limited cutaneous systemic sclerosis (commonly referred to as CREST syndrome- Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasias). Diffuse cutaneous systemic sclerosis tends to involve more proximal skin changes, and can be associated with interstitial lung disease. Limited cutaneous systemic sclerosis generally shows more distal skin changes, can involve the problems listed in the letters “CREST”, and can be associated with primary pulmonary hypertension.
Scleroderma related disorders can be quite severe. However, with appropriate recognition and diagnosis, many of the problems can be anticipated and/or managed. There are newer approaches to treat the most severe forms of disease including interstitial lung disease and primary pulmonary hypertension. These problems are often co-managed with pulmonary physicians and cardiologists.
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