Macroscopic inflammatory tracheal and endobronchial nodules in Sjögren's syndrome

A 38-year-old woman with primary Sjögren's syndrome (SS) presented with a 1 month history of cough and chest pain. She had been treated with prednisolone for organising pneumonia and had been on prednisolone (5 mg/day). Flexible bronchoscopy revealed macroscopic multiple nodules mainly in trachea and in both main bronchus (figure 1A). Three-dimensional CT (3D-CT) also demonstrated multiple nodules in the same place (figure 1B). Endobronchial biopsy showed polyclonal lymphocyte infiltration in the bronchial submucosa and epithelium in part (figure 1C, H & E: upper, CD3: middle, CD20: lower). There were few IgG4-positive plasma cells in the lesion. Amyloid deposition was not detected by direct fast scarlet staining. Abnormal proliferations of squamous epithelium were not observed, and human papilloma virus DNA was not detected in the bronchial lavage fluid. These findings indicated that the inflammatory nodules were associated with SS. Selective cyclo-oxygenase-2 inhibitor, celecoxib, was...