The Clinical Significance of the Anticentromere Antibody

Abstract

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.

Autoantibody Profile in Systemic Sclerosis as a Marker for Esophageal and Other Organ Involvement in Turkish Populations

“…In another study it was suggested that ACA positivity is associated with a more benign form of SSc with limited cutaneous and internal organ involvement. In that study the mean disease duration of ACA-positive patients was found to be markedly longer than that of ACA-negative patients [22].…”

CRST Syndrome (Calcinosis Cutis, Raynaud's Phenomenon, Sclerodactyly, and Telangiectasia) Associated with Autoimmune Hepatitis.

“…At this time, antinuclear antibodies of discrete speckled pattern and anticentromere antibodies were identified. These findings provided clear evidence of CRSTsyndrome (2,12). However, the pattern of liver dysfunction, which consisted of hypergammaglobulinemia, and the elevation of ASTand ALT, had not changed.…”

Lung function abnormalities and decline of spirometry in scleroderma: an overrated danger?

“…Fortyeight patients (49% of those asked) complained of dyspnoea on exertion, 6 of dyspnoea at rest, 20 (24% of those asked) admitted to dry cough and 10 (12%) to productive cough. Smoking status was recorded for 72 patients: 41 (57%) were nonsmokers, 17 (24%) were ex-smokers and 14 (19%) were current smokers at presentation.…”