Constrictive Bronchiolitis in Patients with Primary Sjögren Syndrome

Objective Constrictive bronchiolitis (CB) is a poorly understood pulmonary manifestation of primary Sjögren syndrome (pSS). We aimed to clarify the presenting clinicoradiologic features and clinical course of pSS-associated CB through a retrospective cohort study. Methods We retrospectively identified 11 patients with pSS and CB (defined by mosaic pattern with air trapping on computed tomography) encountered at our institution over 9 years from 2007 to 2015. Presenting clinical features, laboratory results, radiologic findings, and clinical course were analyzed. Results Our patients were mostly women (91%), with a median age 53 years (range, 31-76 years) at the time of pulmonary symptom (dyspnea or cough) onset. Most (64%) were nonsmokers. Average interval between diagnosis of pSS and the onset of pulmonary symptoms was 4.4 years; in 4 patients (36%), CB was the presenting manifestation of pSS. Chest radiographs were normal (67%) or demonstrated hyperinflation (33%). Pulmonary function testing demonstrated airflow obstruction in 7 patients (63%), and 2 patients (18%) had an isolated reduction in diffusing capacity, whereas the remaining 2 patients manifested mixed physiology or normal results. Surgical lung biopsy was performed in 2 patients and confirmed the diagnosis of CB in both. Treatment was variable among our patients included glucocorticoids, hydroxychloroquine, mycophenolate mofetil, and cyclophosphamide. Among 6 patients with follow-up computed tomography and pulmonary function, the results remained stable or worsened. Conclusions Constrictive bronchiolitis is a rare extraglandular manifestation and can sometimes be the presenting manifestation of pSS. This pulmonary manifestation is associated with an indolent clinical course and does not respond well to immunosuppressive therapy.