ABSTRACT
Scleroderma is a rare connective tissue disorder characterized by sclerosis of the skin and subcutaneous tissue along with variable visceral involvement. While in morphea the disease is mainly limited to the skin and underlying tissues, systemic sclerosis is associated with multiple systemic complications and can present as various emergencies. Systemic sclerosis is probably the autoimmune rheumatic disease with the highest disease-related morbidity and mortality and is quite heterogeneous in its clinical presentation, progression, and therapeutic response. Cutaneous involvement in systemic sclerosis includes the edematous phase followed by the indurative phase and finally the atrophic phase. In addition, painful digital ulcers, pitted scars, and Raynaud phenomenon are commonly seen. Interstitial lung disease and pulmonary hypertension are the two primary pulmonary manifestations. Gastrointestinal involvement occurs in the form of motility dysfunction and mucosal damage that occur in different segments of the gastrointestinal tract. Myocardial fibrosis, fibrosis of the conduction system, and pericardial involvement may be seen. Variable degrees of renal and neurological involvement are seen. Generalized inflammatory arthralgias, synovitis, tendon friction rubs, and acroosteolysis characterize musculoskeletal involvement. Various emergencies that can be encountered in systemic sclerosis include digital gangrenes, pulmonary arterial hypertension, interstitial lung disease, scleroderma renal crisis, erosive esophagitis, pericarditis, and myocardial fibrosis.
