• Keigo Hayashi, Haruki Watanabe, Yuriko Yamamura, Yosuke Asano, Yu Katayama, Sumie Hiramatsu-Asano, Keiji Ohashi, Michiko Morishita, Mariko Narazaki, Yoshinori Matsumoto, Ken-Ei Sada, and Jun Wada.
• Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
• Medicine (Baltimore). 2021 Jan 22; 100 (3): e24028.

RationaleBronchial involvement alone is a rare initial manifestation of granulomatosis with polyangiitis (GPA). Herein, we report a case of refractory GPA with obstructive pneumonia caused by bronchial involvement.Patient ConcernsA 65-year-old man complained of a 2-week cough and fever.DiagnosesConsidering the presence of opacities and multiple consolidations in both lungs due to obstruction or stenosis on the bronchus, which did not respond to antibiotics, and proteinase-3-antineutrophil cytoplasmic autoantibody positivity, he was diagnosed with GPA. Positron emission tomography- computed tomography scan revealed no abnormal findings in the upper respiratory tract.InterventionsHe was treated with prednisolone (PSL, 50 mg/d) and intravenous cyclophosphamide.OutcomesHis general and respiratory symptoms improved. However, 8 weeks after PSL treatment at 20 mg/d, he developed a relapse of vasculitis along with sinusitis and hypertrophic pachymeningitis. Hence, PSL treatment was resumed to 50 mg/d, and weekly administration of rituximab was initiated. Consequently, the symptoms gradually mitigated.LessonsGPA with bronchial involvement is often intractable and requires careful follow-up, which should include upper respiratory tract and hypertrophic pachymeningitis assessment.Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

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