STENOSIS OF THE BRONCHIAL TREE DUE TO GRANULOMATOSIS WITH POLYANGIITIS

Abstract

SESSION TITLE: Medical Student/Resident Pulmonary Manifestations of Systemic Disease 1 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Tracheobronchial tree (TBT) stenosis is a severe complication of Granulomatosis with Polyangiitis (GPA) due to granulomatous lesions along the respiratory tract. There is limited data regarding appropriate therapy for these patients. This is a case of a rare subtype of TBT, bronchial stenosis. CASE PRESENTATION: A 73-year-old woman with a history of rheumatic fever, chronic obstructive pulmonary disease requiring tracheostomy with reversal and residual tracheal stenosis, presented with a productive cough worsening over three months. She had associated chills, fevers, night sweats, dyspnea, and weight loss. Her sputum was brown without hemoptysis. She denied prior tobacco use and had no recent sick contacts or travel. Antibiotic and steroid therapy provided no relief. Chest radiograph showed hyperinflation, bilateral patchy infiltrates, and left lower lobe atelectasis. A computerized-tomography scan showed complete impaction of the left main bronchus with collapse of the left upper and lower lobes, significant bronchiectasis, and mediastinal lymphadenopathy.Bronchoscopy revealed 70% stenosis of the upper trachea, friable respiratory mucosa, and complete occlusion of the left main-stem bronchus. The right main-stem bronchus and the bronchus intermedius were circumferentially narrowed by granulation tissue. Cryoablation of the left main stem occlusion was unsuccessful.Endobronchial biopsy revealed granulation tissue with fibrosis and multi-nucleated giant cells. Her cytoplasmic anti-neutrophil cytoplasmic antibody titer was 1:320. Bronchial washings were negative for bacterial organisms. She was then diagnosed with bilateral bronchial stenosis due to GPA. DISCUSSION: TBT stenosis presents in 12-23% of patients with GPA. The stenosis is due to a non-infectious ulcerative tracheobronchitis caused by an underlying small vessel vasculitis, followed by fibrotic scarring. Symptoms such as cough, hemoptysis, or dyspnea mimic infection or obstructive lung disease.There is no clear consensus on the management of patients with bronchial stenosis. These fibrotic lesions are often refractory to pharmacological management with corticosteroids or immunosuppressive agents alone, and an endoscopic intervention such as cryotherapy, balloon dilation, or stent placement is needed for symptomatic relief. The benefits of endoscopic intervention can be transient due to a high restenosis rate. Comparison of patient outcomes based on the treatment modality of choice would prevent delay in therapy or worsening of disease. CONCLUSIONS: Bronchial stenosis is a complication of GPA that can mimic other common obstructive lung diseases due to the similar pathogenesis of bronchoconstriction. Optimal therapeutic approach for bronchial stenosis is controversial. Literature currently supports a combination of pharmacological and endoscopic therapy. Investigation of patient outcomes based on treatment modality is warranted. Reference #1: Girard et al. Tracheobronchial Stenosis in Granulomatosis With Polyangiitis (Wegner’): A Report on 26 Case. Medicine (Baltimore). 2015 Aug;94(32):e1088. Reference #2: Daum et al. Tracheobronchial Involvement in Wegener’s Granulomatosis. Am J Respir Crit Care Med. 1995 Feb;151(2 Pt 1):522-6. Reference #3: Terrier et al. Granulomatosis with Polyangiitis: Endoscopic Management of Tracheobronchial Stenosis: Results From a Multicenter Experience. Rheumatology (Oxford). 2015 Oct;54(10):1852-7. DISCLOSURES: No relevant relationships by Omar Ibrahim, source=Web Response No relevant relationships by Anil Magge, source=Web Response No relevant relationships by Rahul Mutneja, source=Web Response No relevant relationships by Evan Nadler, source=Web Response No relevant relationships by Aysha Tandon, source=Web Response