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© 1987 British Society for Rheumatology

research-article

THE CLINICAL SIGNIFICANCE OF THE ANTICENTROMERE ANTIBODY

MARIAN H. MILLER, G. O. LITTLEJOHN, ANN DAVIDSON, B. JONES and D. J. TOPLISS

Rheumatic Disease Unit, Prince Henry's Hospital Melbourne, Victoria, Australia

Correspondence to: Address correspondence to Dr. G. O. Littlejohn, Department of Medicine, Prince Henry's Hospital, St. Kilda Road, Melbourne 3004, Australia

To elucidate further the clinical significance of the anticentromere antibody (ACA), 32 Australian-born Caucasian patients with scleroderma (SD) or CREST (calcinosis, Raynaud's phenomenon, oesophageal dysmotility, sclerodactyly and telangiectasia) were reclassified as ACA-positive (19 patients) or ACA-negative (13 patients). The clinical features of the two groups were compared. Mean disease duration was 11.7 years for the ACA-positive group and 1.1 years for the ACA-negative group. No ACA-positive patient had generalized skin SD, whereas all ACA-negative patients with disease duration greater than 6 months had moderate or severe proximal scleroderma. Only one ACA-positive patient had serious extra-oesophageal internal organ involvement, excluding primary biliary cirrhosis (three patients) which is itself associated with ACA. Three ACA-negative patients had serious extra-oesophageal internal organ involvement. No ACA-positive patient had been treated with penicillamine or captopril compared with 11 ACA-negative patients. Thus ACA appears to be a favourable prognostic indicator. Analysis of individual CREST manifestations in ACA-positive patients revealed that most had ‘incomplete CREST’, lacking one or two of the five features. The classification of SD patients as ACA-positive or ACA-negative is suggested.

KEY WORDS: Scleroderma, CREST, Anticentromere antibody


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