The sera of 1373 patients derived from a random population of 2627 individuals examined in a rheumatology outpatient clinic were found to be positive for antinuclear antibodies (ANA). Forty-seven of 1373 ANA positive patients (3.42%) presented a pattern of anticentromere antibodies (ACA), and of these 41 were followed-up. The predominant clinical features among ACA positive patients were Raynaud's phenomenon, arthralgias, scleroderma affecting the hands, feet, face and forearms, telangiectases and pulmonary involvement. There were 26 patients (64%) with limited cutaneous systemic sclerosis (lSSc), seven with primary Sjögren's syndrome (Ss) (17%), five with idiopathic Raynaud's phenomenon (IRPh) (12%) and three (7%) with other diseases. The lSSc subset was characterized by a significantly younger age for disease onset than for the IRPh patients. The Raynaud's phenomenon of lSSc patients lasted for many years before the onset of scleroderma, while their pulmonary involvement was mild and subclinical. The Ss patients with ACA were characterized by a lower incidence of parotid gland enlargement and anti-La (SSB) antibodies than described in the literature. Finally, the presence of ACA correlates with the clinical criteria proposed for the diagnosis of lSSc.