Constrictive (Obliterative) Bronchiolitis in Primary Sjögren Syndrome

Abstract

SESSION TITLE: Systemic Disease and Pulmonary Manifestations SESSION TYPE: Original Investigation Poster PRESENTED ON: Wednesday, November 1, 2017 at 01:30 PM - 02:30 PM PURPOSE: Extraglandular involvement in Sjögren’s syndrome (SS) includes a broad spectrum of lung diseases. Constrictive (obliterative) bronchiolitis (CB) is a poorly understood pulmonary manifestation of SS. The purpose of this investigation is to understand the clinico-radiologic features and clinical course of primary SS-associated CB. METHODS: We retrospectively identified 11 patients with primary SS and constrictive bronchiolitis encountered at our institution during a 9-year period from 2007-2015. Clinical features, laboratory results and radiologic findings were analyzed. Treatment data was extracted and correlated with follow-up course including imaging and pulmonary function testing. RESULTS: Our patients were mostly women (91%), with a median age of 50 years (range, 22 to 76 years) at the time of SS diagnosis and 53 years (range, 31 to 76 years) at the time of pulmonary symptom onset. Average interval between diagnosis of SS and the onset of pulmonary symptoms was 4.4 years; four patients (36%) were diagnosed with SS after the onset of pulmonary symptoms. Most patients (64%) were nonsmokers. The majority of patients had positive serology to anti-nuclear antibodies (82%), rheumatoid factor (55%), anti-Ro/SS-A (82%) and anti-LA/SS-B (64%) antibodies. Patients most frequently presented with dyspnea (91%) and cough (36%). Chest radiographs were normal (67%) or demonstrated hyperinflation (33%). High-resolution CT chest revealed mosaic attenuation with air trapping in all patients. PFT demonstrated airflow obstruction in 7 patients (63%), 2 patients (18%) had an isolated reduction in diffusing capacity, 1 (9%) had mixed physiology and the remaining patient (9%) had normal PFT results. Surgical lung biopsy was performed in two patients and confirmed the diagnosis of CB; one additional patient underwent bronchoscopic lung biopsy which yielded nondiagnostic findings. Treatment was variable and included glucocorticoids, hydroxychloroquine, mycophenolate mofetil, and cyclophosphamide. Most patients remained stable or declined during follow-up (median 8 months; range 0-160); one patient treated with hydroxychloroquine and mycophenolate mofetil demonstrated improvement. There were no deaths or lung transplants. CONCLUSIONS: CB is an uncommon pulmonary manifestation in primary SS and manifests mosaic attenuation with air trapping on chest CT scan, usually associated with evidence of airflow obstruction on PFT. Optimal therapy remains to be identified. CLINICAL IMPLICATIONS: Constrictive bronchiolitis can be seen in patients with primary SS and can be identified by the presence of mosaic pattern with air trapping on chest CT. DISCLOSURE: The following authors have nothing to disclose: Elizabeth Wight, Misbah Baqir, Jay Ryu No Product/Research Disclosure Information