RESUMEN
BACKGROUND: Anti-centromere autoantibodies (ACA) are frequently detected in systemic sclerosis (SScl), especially in the calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia (CREST) syndrome, in which a prevalence of 55% has been reported. The presence of ACA in systemic lupus erythematosus (SLE) is so rare that its detection can raise serious doubts about the validity of the diagnosis. OBJECTIVE: To determine the frequency of ACA positive subjects from a wide monocentric cohort of SLE patients and analyse the clinical and biological characteristics of this group. METHODS: Five hundred and sixty consecutive SLE patients were systematically analysed for the presence of ACA and other autoantibodies using indirect immunofluorescence, counter-immunoelectrophoresis, double immunodiffusion, enzyme-linked immunosorbent assay (ELISA), and Western-blot. RESULTS: ACA were detected in 11 SLE patients (1.9%); all of them were women. The CENP-B-specific ELISA was positive in all patients. The main clinical features of scleroderma (cutaneous sclerosis, sclerodactylia, digital ulcers, or pulmonary fibrosis) were not present in these patients, who did not differ clinically from the whole SLE group. CONCLUSIONS: ACA can be detected in patients with genuine SLE without concurrent scleroderma. Therefore, the presence of this antibody does not preclude the possibility of the diagnosis of SLE. In addition, SLE patients with ACA do not represent a different clinical subgroup.