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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 …