Clinical approach to scleroderma

Systemic sclerosis (SSc) is a heterogenous disease with a morbidity and mortality that varies widely. Nonetheless, the future clinical course of an individual patient can be estimated based on the severity of skin and internal organ involvement within the first several years of the disease. Patients with limited cutaneous SSc (ISSc) have skin thickening below the elbows or knees and may have face and neck involvement. Patients with this subtype of SSc have Raynaud's phenomenon, digital ulcers, and esophageal dysfunction. Significant morbidity and mortality arises in those patients with ISSc who develop interstitial lung disease or pulmonary artery hypertension. Patients with diffuse cutaneous SSc (dSSc) have skin thickening above the elbows and knees or on the trunk. These patients have a more abrupt onset of disease, often with constitutional symptoms and arthalgias. Severe heart, lung, gut, and renal involvement, if it occurs, tends to develop within the first 5 years of disease, especially within the first several years. Patients with significant internal organ involvement have a poorer prognosis than patients who do not. The goals of the initial history and physical and laboratory examinations are to classify the type of scleroderma as ISSc or dSSc, to estimate disease duration, and to define the extent and severity of organ involvement. Treatment of SSc is organ based. Treatment may reduce morbidity associated with Raynaud's phenomenon, digital ulcers, esophageal dysmotility, esophageal reflux, gut dysmotility, arthralgias, myositis, and pulmonary artery hypertension. Therapy may stabilize lung function in patients with interstitial lung disease with alveolitis and stabilize renal function in patients with renal crisis. The overall prognosis for patients with SSc appears to be improving. Patients with early dSSc should be considered for enrollment onto protocol testing of potential disease-modifying therapies.