A case of cellular bronchiolitis with primary Sjögren syndrome
Kazue Saitoa Yoshinori Taninoa Suguru Satoa Kazuhiro Tasakib Akira Hebisawac Mitsuru Munakataa
aDepartment of Pulmonary Medicine, School of Medicine, Fukushima Medical University
bDepartment of Pathology, Fukushima Medical University Hospital
cDivision of Clinical Laboratory, National Hospital Organization Tokyo National Hospital
A 67-year-old woman was referred to our hospital because of progressive productive cough, dyspnea on exertion, and abnormal chest shadows. She had been diagnosed as diffuse panbronchiolitis and has been treated with macrolides with no improvement. Chest computed tomography (CT) revealed bilateral diffuse centrilobular nodules, consolidation, and bronchiectasis. The diagnosis of primary Sjögren syndrome was made by xerostomia, positive serum SS-B antibody, and positive Schirmer test without symptoms, which suggest other collagen vascular diseases. Lung biopsy using video-associated thoracoscopy revealed transmural cellular infiltrate of small round cells around the entire circumference of bronchioles, and a pathological diagnosis of cellular bronchiolitis was made. Her symptoms and small nodular shadows on CT were improved after a 3-month treatment of prednisolone. We should take cellular bronchiolitis into account as one of the pulmonary manifestations of primary Sjögren syndrome because the bronchiolar disorder may be improved by steroid therapy.
Primary Sjögren syndrome Cellular bronchiolitis IgG4-related lung disease
Received 9 Feb 2011 / Accepted 20 Jan 2012
AJRS, 1(4): 304-310, 2012