New ACR EULAR guidelines for systemic sclerosis classification.

Abstract

The American College of Rheumatology and European League Against Rheumatism classification criteria for systemic sclerosis are a significant advancement in the field. This article describes the innovative, rigorous, criteria development strategy that was used. The new criteria build upon previous criteria by incorporating important elements (proximal scleroderma, sclerodactyly, digital pits, pulmonary fibrosis, Raynaud's phenomenon, and scleroderma specific autoantibodies). The new criteria add emphasis to the vasculopathic manifestations, and include the early manifestation of puffy fingers. Together, these enhancements have resulted in a shift in the conceptual framework of the disease for the next generation. The new criteria have improved sensitivity and specifically, particularly among cases with early disease, mild disease, or limited disease. The ability to classify more cases, at an earlier stage, may confer the opportunity to intervene and prevent disease progression. Undoubtedly, this will lead to a paradigm shift in the conduct of clinical trials in systemic sclerosis.