A Case of Granulomatosis with Polyangiitis Presenting with Bronchus Ulceration

Cheng-Te Wang ； Ching-Yao Yang

《胸腔醫學》 35卷3期 (2020/09) Pp. 147-151

摘要

Granulomatosis with polyangiitis (GPA), formerly known as Wegner's granulomatosis, is a form of systemic vasculitis that involves primarily the upper and lower airways and the kidneys. The most frequent airway manifestations include subglottic stenosis and inflammation, and tracheal and bronchial stenosis, while ulcerations in tracheobronchial mucosa are uncommon findings. Here, we reported the case of a 77-year-old man who presented with cough, hemoptysis, and progressive joint pain in all 4 limbs for about 2 months, accompanied with intermittent low-grade fever in the evening for 2 weeks. Bronchial ulcerations were found by bronchoscopy, and biopsy yielded a non-specific granulomatous inflammation without positive staining for relevant pathogens. Due to a contact history with tuberculosis patients and unresolved fever despite antibiotics treatment, the tracheobronchial granulomatous inflammation was diagnosed as tuberculosis at first. However, his symptoms did not improve in spite of empirical anti-tuberculosis antibiotics treatment. Later, other symptoms developed, including otitis media and acute glomerulonephritis, which manifested with hematuria and acute kidney injury. The serological test revealed elevation of cytoplasmic antineutrophil cytoplasmic antibodies. Thus, the patient was diagnosed with GPA with airway, ear, and kidney involvement. After immunosuppressive therapies with cyclophosphamide and glucocorticoid for GPA, his symptoms improved, and the follow-up bronchoscopy showed resolution of the bronchial ulcerations.

關鍵字

granulomatosis with polyangiitis ； bronchus ulceration ； endobronchial tuberculosis