Constrictive Bronchiolitis in Patients With Primary Sjögren Syndrome

Abstract

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.

Improvement of constrictive bronchiolitis (bronchiolitis obliterans) after rituximab therapy in 2 patients with primary sjögren syndrome

“…Constrictive bronchiolitis, a subtype of obstructive small-airway disease, affects a minority of patients with primary SS [ 5 ] and is characterized by progressive airflow limitation. No effective treatment is currently available.…”

“…For our patients, cough was 1 of the main presenting symptoms and was accompanied by wheezing, which caused both patients to be mistakenly diagnosed with asthma. Prior studies have also described dyspnea with cough and bronchial responsiveness to be predominant subjective features of patients with primary SS and pulmonary involvement [ 5 , 7 ].…”

“…SS can occur as a primary disease (primary SS) or in association with other autoimmune diseases (secondary SS) [ 4 ]. Pulmonary involvement occurs in over half of patients who may or may not have associated symptoms [ 5 , 6 ]. Findings in secondary SS are often confounded by the pulmonary disease from the underlying connective tissue process [ 7 ].…”

“…The small-airway involvement has primarily been characterized histologically by 2 types of bronchiolitis: follicular and constrictive [ 9 , 10 ]. The latter is associated with worsening airflow obstruction despite treatment with bronchodilators, macrolides, inhaled and systemic glucocorticoids, and other glucocorticoid-sparing agents [ 5 , 7 ]. Several reports indicated improvement in symptoms, radiographic findings, and pulmonary function when rituximab was used for patients with SS-related interstitial lung disease [ [11] , [12] , [13] ].…”

Bronchiolitis and Bronchiolar Disorders

“…Cryptogenic constrictive bronchiolitis is rare, predominantly presenting as persistent cough and worsening dyspnea, and occurs mostly among never-smoking middle-aged and older women. 12,35,37 The existence of cases of cryptogenic bronchiolitis was first considered by Turton et al 146 Kindt et al, 147 in Fig. 16 RB-ILD.…”