The Many Signs of Scleroderma: How to Make a Differential Diagnosis - PracticalPainManagement.com
Scleroderma, or systemic sclerosis, remains the most challenging rheumatic disease to treat and holds the highest mortality rate. This review explores the unique pathophysiology, clinical features, and therapeutic approaches to this systemic connective tissue disease. During my near half-century of rheumatology practice, I found that scleroderma, or systemic sclerosis, to be the most challenging rheumatic disease to diagnose and manage. Its pathogenesis is less well-understood than other systemic connective tissue diseases – such as systemic lupus erythematosus (SLE) or rheumatoid arthritis (RA) – and this gap has impeded development of effective therapies. Further impacting management is the fact that systemic sclerosis (SSc) presents in a variety of ways, often resulting in consultation with myriad specialists, diagnostic confusion, and treatment delays. In this review, the many presentations of SSc will be illustrated with hypothetical case examples, as a means to exploring the di