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Pathology General b54af3d2

More than 90% patients of CREST syndrome with the limited cutaneous form of this disorder make which of the following autoantibodies?

A
Anti-centromere
B
Anti-DNA topoisomerase l
C
Anti-double-stranded DNA
D
Anti-Golgi
High-Yield Explanation
All forms of scleroderma are thought to have a strong autoimmune component, and glucocoicoids and azathioprine are used to suppress the inflammatory complications of scleroderma. (Other drugs that can be used in therapy include penicillamine, which inhibits collagen cross-linking, NSAIDS for pain, and ACE inhibitors to protect the kidney if hypeension or renal damage occurs). The anti-centromere antibody is quite specific for CREST syndrome (96% of cases), and is only seen in a minority of patients with diffuse scleroderma (mainly those with Raynaud's phenomenon) and rarely in systemic lupus erythematosus and mixed connective tissue disease.Also know:Anti-DNA topoisomerase I , also called anti-Scl-70 occurs commonly (64-75%) in diffuse scleroderma, but only rarely in CREST syndrome.Anti-double-stranded DNA is fairly specific for systemic lupus erythematosus, although it only occurs in 50-60% of lupus cases.Anti-Golgi antibodies are seen most often in systemic lupus erythematosus and Sjogren syndrome. Ref: Hellmann D.B., Imboden Jr. J.B. (2013). Chapter 20. Musculoskeletal & Immunologic Disorders. In M.A. Papadakis, S.J. McPhee, M.W. Rabow (Eds),CURRENT Medical Diagnosis & Treatment 2013.

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