B35-49 A Case of Granulomatosis With Polyangiitis Complicated by Left Mainstem Bronchus Obstruction

Abstract Granulomatosis with polyangiitis (GPA) is a rare necrotizing, noncaseating granulomatous vasculitis which affects the small and medium sized vessels. Pulmonary manifestations are well documented, however bronchial involvement is rare. Case A 54-year-old Japanese female with no known past medical history presented to the hospital with persistent fevers. She was discharged from the hospital one week prior for dyspnea secondary to influenza A and community acquired pneumonia, and completed a course of antibiotics and oseltamivir outpatient. A week prior to this, she completed a course of antibiotics for bilateral otitis media. She reported daily fevers, chills, sinus congestion and generalized weakness, prompting her return to the emergency department. Computed tomography (CT) of the chest showed a large left lower lobe mass-like consolidation and numerous pulmonary nodules in the right lower lobe. A bronchoscopy was performed, however there was significant airway narrowing and the diagnostic bronchoscope was unable to pass the distal left mainstem bronchus. The airway mucosa was markedly abnormal, friable and diseased, with easy mucosal bleeding. Bronchial washings and biopsies were obtained. Vasculitis studies were ordered which revealed cytoplasmic anti-neutrophil cytoplasmic antibodies (c-ANCA) titer of 1:80, high proteinase 3 (PR3) titer of 71.9, as well as low-positive SS-A and SS-B antibodies. A CT-guided lung biopsy was obtained which showed extensive areas of basophilic necrosis, granulomatous inflammation, and neutrophilic infiltration of the medium-sized vessel wall. Overall, her clinical picture was consistent with granulomatosis with polyangiitis, and high dose steroids were initiated after discussion with rheumatology. The patient’s symptoms improved and she was discharged with oral steroids and close rheumatology follow up. Unfortunately, the patient presented with hemoptysis and epistaxis at her outpatient rheumatology follow up appointment, and was admitted to a tertiary care hospital for a vasculitis flare. She received pulse dose steroids with rituximab and recovered, and was discharged home. Discussion GPA is a multisystem vasculitis which is characterized by renal, pulmonary, otic and sinus involvement. c-ANCA are highly specific for GPA. Histologic evidence of necrotizing vasculitis can assist with diagnosis. Tracheobronchial stenosis (TBS) is a life threatening manifestation of GPA, and responds poorly to corticosteroids, with most patients experiencing relapse. Treatment of GPA is typically achieved with corticosteroids and immunosuppressive drugs including cyclophosphamide, however rituximab may be more effective in inducing remission as noted in recent studies. Prompt laboratory studies, bronchoscopy and histologic investigation can lead to early initiation of therapy for this lethal disease. This abstract is funded by: None