전신경화증의 새로운 분류기준

Abstract

Systemic sclerosis (SSc) is a connective tissue disease of unknown origin, which is characterized by fibrosis of the skin and internalorgans, and endothelial and immunologic dysfunction. The presence of a wide range of symptoms renders disease classificationdifficult. Although recent studies have contributed to our understanding of this debilitating illness, well-validated classificationcriteria are required for accurate comparison between registries and clinical trials, to assess response to treatment, morbidityand prognosis. Given the emphasis placed upon early and aggressive treatment, the 1980 American College of Rheumatology(ACR) classification criteria are of limited utility with respect to early diagnosis of SSc and limited cutaneous SSc. Recently, the2013 ACR/European League Against Rheumatism classification criteria for SSc were published for research and clinical practicepurposes. These criteria include skin thickening, fingertip lesions, telangiectasia, abnormal nailfold capillaries, Raynaud’s phenomenon,SSc-specific autoantibodies and pulmonary complications pertaining to vasculopathy, autoimmunity and fibrosis. These updatedcriteria should allow a greater number of patients to receive an early diagnosis of SSc.