Pulmonary AL amyloidosis in a patient with primary Sjögren syndrome.

Abstract The condition of a 29-year-old woman with primary Sjögren syndrome (SS) was complicated by amyloid light chains- (AL-) type amyloidosis in the paranasal sinus. She had not complained of respiratory symptoms, but her chest computed tomography (CT) scan revealed bilateral multiple nodular shadows. Lung biopsy specimens using video-associated thoracoscopy showed amyloidoma in a subpleural nodular lesion and amyloid deposits in the interstitial parenchymal walls and pulmonary vessels. Pulmonary AL amyloidosis, presumably related to a chronic inflammatory lymphoproliferative process in SS, has rarely been reported.