Pulmonary Manifestations of Systemic Disease: Pulmonary Manifestation of Systemic Disease |

Tracheobronchial Manifestations and Bronchoscopic Therapies in Patients Diagnosed With Granulomatosis With Polyangiitis FREE TO VIEW

Hanine Inaty, MD; Anupam Kumar, MD; Aparna Das, MD; Joseph Cicenia, MD; Francisco Almeida, MD; Sonali Sethi, MD; Thomas Gildea, MD; Michael Machuzak, MD
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Cleveland Clinic, Cleveland, OH

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

Chest. 2016;150(4_S):1067A. doi:10.1016/j.chest.2016.08.1174
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SESSION TITLE: Pulmonary Manifestation of Systemic Disease

SESSION TYPE: Original Investigation Poster

PRESENTED ON: Wednesday, October 26, 2016 at 01:30 PM - 02:30 PM

PURPOSE: Granulomatosis with polyangiitis (GPA) is largely a systemic disease, with the lungs being the most commonly affected organs. Although it can affect the airways in up to 15-55% of cases, tracheobronchial disease remains under recognized, with paucity of published data. The aim of this study is to describe airway disease manifestations and various bronchoscopic therapies implemented in a large volume referral center.

METHODS: Retrospective review of charts of patients diagnosed with GPA who underwent bronchoscopies between January 2015 and January 2016.

RESULTS: 23 patients with an established diagnosis of GPA underwent a total of 103 bronchoscopies. Female: Male ratio was 3:1, with a mean age of 41 years. 83% of patients were on immunosuppressive therapy at the time of bronchoscopy. The majority of patients were symptomatic (77%) with dyspnea (74%), cough (65%) and wheezing (37%) being the most common symptoms at presentation. On bronchoscopic examination, airway disease was identified in all patients, most commonly at the level of the subglottis (64%), trachea (51%) and distal airways (75%), while disease of the vocal cords was rarely seen (7%). The most common types of airway abnormalities included multifocal tracheobronchial stenosis (75%), subglottic stenosis (59%) and localized strictures (31%). Mucosal abnormalities such as edema, ulcerations and cobble stoning were seen in 36% of airway examinations. Other rare disease manifestations such as synechial membranes, pseudopolyps and submucosal tunnels were also observed. Additionally, thick crusty secretions were a common finding (39%). Endobronchial biopsies, when performed, showed no evidence of vasculitis. Notably, various bronchosopic interventions were effective at treating airway obstruction due to GPA. Balloon dilation was most commonly used (67%), along with steroid injections (63%) and application of mitomycin (39%). Other techniques included: rigid dilations (10%), electrosurgery (28%), cryotherapy (28%) and stent placement (14%). Improvement of airway disease was seen on 65% of repeat bronchoscopies. Moreover, symptoms improved in 89% of patients post intervention. No procedural complications or deaths were reported.

CONCLUSIONS: Tracheobronchial disease is a common cause of morbidity in patients with GPA. It has various bronchoscopic manifestations, which can be at times subtle. Bronchoscopy plays an essential role in early diagnosis and therapy.

CLINICAL IMPLICATIONS: Airway abnormalities can progress into scarring and stenosis despite appropriate immunosuppressive therapy. Various bronchoscopic techniques are effective in controlling the disease and providing immediate relief of symptoms.

DISCLOSURE: The following authors have nothing to disclose: Hanine Inaty, Anupam Kumar, Aparna Das, Joseph Cicenia, Francisco Almeida, Sonali Sethi, Thomas Gildea, Michael Machuzak

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