Bronchiolitis obliterans with organized pneumonia, a rare complication of primary Sjogren's syndrome.

Introduction. - Bronchiolitis obliterans organizing pneumonia (BOOP) is cha racterized by plugs of granulation tissue in bronchioles, alveolar ducts an d alveoli This pulmonary disorder has been reported in some cases in relati on to drug consumption (D-penicillamine, amiodarone), with bacterial or vir al infections (Mycoplasma pneumoniae, HIV), and with systemic diseases, suc h as rheumatoid arthritis. To our knowledge, only three cases of associatio n BOOP-Sjogren's syndrome have been reported. Exegesis. - We report three new cases of BOOP. These patients presented a p rimary Sjogren's syndrome without clinical or biological abnormalities sugg estive of other autoimmune diseases. Initial presentation was an acute pulm onary disorder mimicking a bacterial pneumonia. Two patients had cutaneous vasculitis and the third vasculitic neuropathy. Corticosteroid therapy was begun and was quickly successful. None of the patients presented a relapse of BOOP. Conclusion. - The incidence of BOOP is probably underestimated in patients with primary Sjogren's syndrome without cutaneous vasculitis. In case of pn eumonia with antibiotic resistance, an immunological mechanism should be co nsidered. (C) 2000 Editions scientifiques et medicales Elsevier SAS.