Systemic Sclerosis

Michael H. Weisman, MD, Consulting Editor

Systemic Sclerosis (SSC), of all of the rheumatic diseases, is clearly the most challenging in terms of early diagnosis, understanding its pathogenesis, managing the different skin and internal organ phenotypes, and predicting prognosis. Today even the words renal crisis and scleroderma lung disease frighten the clinician because they know that patients die despite their best efforts to date. Nowhere is this truer than in the quest for its genetic associations where studies have been bedeviled by the lack of agreement regarding deep phenotype characterizations of study subjects. Stepping up to this challenge is Dr Maureen Mayes, who has assembled a strong group of experts in the field to bring the reader up-to-date on significant advances in this complex disease. These reviews focus on recognizing and identifying the clinical manifestations of disease, the mechanisms involved in SSC pathogenesis, the most recent findings regarding discovery of genetic susceptibility markers, and up-to-date management of the common and rare complications of this challenging disease. Maureen and her colleagues are certainly up to the task.

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