We present two cases of Obliterative Bronchiolitis (OB) in the setting of Sjogren's syndrome who were treated successfully with Rituximab (RTX). A 51 yrs. old nonsmoker female came with dry mouth and dry eyes with history of diffuse rashes on her body. Serology came positive for SSAantibody. Lower lip biopsy confirmed the diagnosis of Sjogren's syndrome. She had history of recurrent pneumonia and occasional wheezing. Her pulmonary function tests showed moderate obstruction with hyperinflation and normal diffusing capacity. Exhaled nitric oxide was within normal limits. Her computer tomography (CT) scan of the chest showed diffuse mosaic attenuation with tree-in-bud pattern mainly in the periphery of lower lobes bilaterally. A bronchoalveolar lavage came back negative for any infectious etiology. Patient was treated with RTX that caused marked improvement in her symptoms, CT scan chest findings as well as pulmonary function tests (mild obstruction with air trapping). Another 55 years old female was diagnosed with Sjogren's syndrome about twenty years back based on positive SSA serology and dry mouth and dry eyes. She came with respiratory symptoms of cough and wheezing. She underwent surgical lung biopsy consistent with chronic bronchiolitis. CT scan of the chest showed diffuse mosaic attenuation with decreased vasculature in low attenuation regions compared to adjacent ground-glass opacities. She also had scattered cysts in her lungs. CT scan was consistent with lymphocytic interstitial pneumonitis and OB. Azathioprine was tried in the past but she could not tolerate it. Her lung functions were consistent with very severe obstruction with air trapping and moderate reduction in diffusing capacity. Patient was treated with RTX with significant improvement in her symptoms as well as lung functions especially diffusing capacity increased by 15%. In this patient as the B cells started to reconstitute her pulmonary function started to decline requiring re-treatment with RTX. Bronchiolitis in the setting of Sjogren's disease can present as of two types: follicular and obliterative. OB, in general, has a varied clinical course, but in most settings tends to be progressive and poorly responsive to corticosteroids/immunosuppresants therapy. In another study of 78 patients with Sjogren's disease, one had bronchial involvement with a favorable response to RTX. Although the data on the use of rituximab for OB in the setting of Sjogren's disease is very limited, RTX should be considered in such cases.

Constrictive bronchiolitis is one of the manifestations of small-airway involvement in primary Sjögren syndrome (SS) and is associated with fixed airflow obstruction despite treatment with bronchodilators, macrolides, corticosteroids, and corticosteroid-sparing agents. Reports have shown a beneficial effect of rituximab on interstitial lung disease associated with SS, but the effect of rituximab on constrictive bronchiolitis is unknown. Herein, we present 2 cases of patients with constrictive bronchiolitis associated with SS who experienced symptomatic improvement and stabilization of pulmonary function testing (PFT) after rituximab therapy. Lung function declined in one of the patients when B cells reconstituted, with improved PFT results on re-administration of rituximab. Our case reports suggest that B cells may be involved in the pathogenesis of SS-associated constrictive bronchiolitis. Therapy targeting B cells may therefore be helpful in treating this debilitating and refractory condition. Further research is warranted.