Critical Care: Inflammatory Diseases Affecting the Lung |

A Unique Case of Follicular Bronchiolitis FREE TO VIEW

Jeffrey Bonenfant, DO; Aarti Mittal, DO
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University of California Riverside, School of Medicine, Riverside, CA

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):299A. doi:10.1016/j.chest.2016.08.312
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SESSION TITLE: Inflammatory Diseases Affecting the Lung

SESSION TYPE: Student/Resident Case Report Slide

PRESENTED ON: Monday, October 24, 2016 at 11:00 AM - 12:00 PM

INTRODUCTION: Follicular bronchiolitis (FB) is a rare lymphoproliferative disease involving polyclonal lymphoid hyperplasia isolated to small respiratory airways. Case reports have shown associations with connective tissue disorders and immunodeficiency. High resolution computed tomography (HRCT) characteristics are centrilobular nodules, peribronchial cuffing, and ground glass opacities. Although there is no established treatment course, recent literature reports long term macrolide therapy to be effective. We report a case of FB due to Sjogren’s syndrome with dramatic improvement after steroid therapy.

CASE PRESENTATION: A 42 year old woman with Sjogren’s syndrome presents with progressive dyspnea, subjective fevers and night sweats. She denied weight loss, cough, or hemoptysis. CT angiography showed diffuse peripheral reticulonodular pattern, ground glass opacities, multiple thin-walled cysts and mild tree-in-bud changes without lymphadenopathy. Video-assisted thorascopic surgery with biopsy of the right upper and middle lobes with histopathologic findings of prominent lymphoid infiltrate, predominantly peribronchiolar with marked plasmacytosis. These findings were initially concerning for mucosa-associated lymphoid tissue-derived lymphoma. After further review, a diagnosis of follicular bronchiolitis was made. After four weeks of oral steroid therapy, there was marked clinical and radiological improvement. Given the increased risk of lymphoma in Sjogren’s syndrome, in addition to her family history of Hodgkin’s lymphoma and the concerning histopathological findings, the patient was started on rituximab for further treatment.

DISCUSSION: FB is a subcategory of lymphoproliferative disease with a differential that includes lymphoma. This is a case of secondary FB due to the association with a connective tissue disorder. The disease often presents in the fifth decade with progressive dyspnea. Connective tissue disorders likely predispose to antigenic stimulation of polyclonal lymphoid follicles with germinal centers located in the small airways. Immunodeficiency in T-regulatory cells may play a role in worsening disease progression. Treatment in secondary FB is thought to be best directed at the underlying condition. Steroid therapy has been proposed as a bridge to treatment with rituximab. Improvement in symptoms may be variable. Further studies are necessary before a consensus on treatment can be made. Until then, each case remains best treated on an individual basis.

CONCLUSIONS: Follicular bronchiolitis is a rare lung disease associated with Sjogren's syndrome. Currently, there is no standard treatment modality. Our patient recieved high dose steroid therapy followed by rituximab, with marked improvement in imaging and symptoms.

Reference #1: Aerni et al. “Successful Treatment of Follicular Bronchiolitis with Macrolide” Chest. (2005) 128(4_MeetingAbstracts):428S.

Reference #2: Tashtoush et al. “Follicular Bronchiolitis: A Literature Review” J Clin Diagn Res (2015) 9, OE01 - OE05.

DISCLOSURE: The following authors have nothing to disclose: Jeffrey Bonenfant, Aarti Mittal

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