Systemic Sclerosis - Scleroderma

Systemic sclerosis (SSc), also called scleroderma, is a chronic autoimmune disorder characterized by fibrosis of the skin and various organs.   Early diagnosis and classification are important so that patients can be evaluated for organ involvement and/or damage.  However, because SSc is a heterogeneous disease, clinical presentation and disease course vary, and manifestations may overlap with those of other rheumatic disorders, all of which can complicate diagnosis.    Clinical subsets of SSc include limited and diffuse cutaneous disease; in addition, some individuals have SSc overlap syndromes, which involve features of different systemic autoimmune rheumatic diseases (SARDs) in addition to those of SSc.  Autoantibody formation is characteristic of SSc, and laboratory testing involves assessment for SSc-specific autoantibodies.  Criteria antibody tests for the disease include tests for anti-Scl-70 (also known as antitopoisomerase 1), anticentromere antibody (ACA), and anti-RNA polymerase III.  Testing for other antibodies associated with SSc may be indicated if criteria test results are negative. See Laboratory Testing below.

Tabs Content
Content Review: 
January 2019

Last Update: November 2019