Sjogren's syndrome and necrotizing sarcoid-like granulomatosis.

We report the first description of a Sjogren's syndrome associated with nec trotizing sarcoid-like granulmatosis. A 62-year old woman was admitted for diagnostic exploration of fatigue, weight loss, and fever at 38 degrees C w hich had progressed for more than 3 months. Chest X-ray showed several pulm onary opacities. There was a history of tuberculosis and thyroiditis at the age of 20 years. Physical examination revealed a sicca syndrome. There was no objective evidence of respiratory disease. Her heart and chest were cle ar on auscultation. The patient had autoantibodies for SSa and SSb specific antigens. Minor salivary gland biopsy gave a score of 3 in the Chisholm cl assification. Chess X-ray and CT-scan showed diffuse infiltrative opacities which were dense and peripheral with no retraction. Bronchoalveolar lavage , and bronchial and transbronchial biopsies were non-specific. Surgical lun g biospy showed typical aspects of necrotizing sarcoid-like granulomatosis. After high-dose pulse corticosteroid therapy, the opacities disappeared wi th no recurrence under oral steroids at three years.