Update on systemic sclerosis.

Systemic sclerosis (SSc) is a rare autoimmune disease characterized by autoantibody production, small-vessel vasculopathy, and skin and other organ fibrosis. The disease is clinically heterogeneous with most patients having some degree of skin sclerosis with varying organ system involvement. Early disease can be difficult to diagnose, especially with minimal skin sclerosis and absence of anti-nuclear antibody (ANA) positivity; however, studies have demonstrated early diagnosis is important as early treatment could potentially lead to better outcomes. New classification criteria have recently been published that have higher sensitivity for detecting early disease thus allowing for a broader spectrum of patients to be represented in clinical trials. Treatment guidelines have been published based on a limited number of randomized-controlled clinical trials; however, there are ongoing phase II and III clinical trials with novel agents that are promising and will change the treatment landscape over the next decade.