The association of cryptogenic organizing pneumonia (COP) with primary Sjogren's syndrome (PSS) is extremely rare. We report a case of simultaneous diagnosis of PSS and COP. A 70-year-old female presented with fever, non-productive cough and dyspnea of 2 months' duration. She had experienced sicca symptoms for the past 2 years. The chest radiograph revealed a right lower lobe infiltrate, which was unresponsive to antibiotics. Bronchoscopy, bronchoalveolar lavage and an open lung biopsy established the diagnosis of COP, while a lip biopsy was consistent with PSS. The patient improved on steroids. Organizing pneumonia may be one of the early manifestations of PSS. Exclusion of PSS should be part of a thorough evaluation of the patient with COP.
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