Abstract

SESSION TITLE: Pulmonary Manifestations of Systemic Diesase SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Respiratory tract involvement is not typically affiliated with Crohn's disease. Very few cases in literature have documented this association, and it is unknown if the respiratory symptoms correlate with GI symptomatology. It is thought that the large airway involvement is typically responsive to inhaled and systemic steroids. CASE PRESENTATION: 21 year old female with a history of Crohns disease diagnosed at the age of 11 presented with shortness of breath and hoarseness. She was found to have tracheal stenosis that over the course of the next several years was recurrent requiring repeated dilations and was also associated with bronchial stenosis bilaterally also requiring multiple dilation procedures. Biopsies showed acute inflammation with neutrophils, lymphoplasmacytic infiltrate, often with granulation tissue and occasional giant cells. Her respiratory involvement did not correlate with her GI symptoms and required multiple prolonged courses of oral steroids. Complicating the picture was a label of GPA due to sino-nasal disease but repeated ANCA levels were negative and rheumatology deemed her large airway disease to not be secondary to GPA. DISCUSSION: Large airway involvement in inflammatory bowel disease is rare and only a few cases have been reported in literature, the first in 1976. Typically the timeline and severity of airway involvement is independent of GI involvement from the disease. Important differentials to rule out are sarcoidosis, granulomatous polyangiitis, and tuberculosis. Noncaseating granulomas are rarely seen in biopsies which usually reveals neutrophilic, lymphoplasmacytic and macrophage infiltration. In more severe cases, tracheobronchial stenosis can occur although the large airway disease generally responds well to inhaled and systemic steroids. In our case the patient required multiple dilation procedures as well as prolonged steroids; at times in the intravenous form at high doses. CONCLUSIONS: Large airway involvement is a rare manifestation of Crohn's disease and doesn't necessarily respond to inhaled steroids and at times warrants long term high dose systemic steroid therapy as well as repeated tracheobronchial dilation procedures. Reference #1: Seunghyun Park, Jongha Park, Hyun-Kuk Kim et al. Tracheal Involvement in Crohn Disease: the First Case in Korea. Clin Endosc. 2016 Mar; 49(2): 202–206. Reference #2: Kuźniar T, Sleiman C, Brugière O, et al. Severe tracheobronchial stenosis in a patient with Crohn’s disease. Eur Respir J. 2000;15:209–212 Reference #3: Plataki M, Tzortzaki E, Lambiri I et al. Severe airway stenosis associated with Crohn’s disease: case report. BMC Pulm Med. 2006;6:7 DISCLOSURES: No relevant relationships by Hina Ahmed, source=Web Response

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