Open AccessGranulomatosis with polyangiitis with obstructive pneumonia progressing to hypertrophic pachymeningitis
Author(s) -
Keigo Hayashi,
Hirotaka Watanabe,
Yuriko Yamamura,
Yosuke Asano,
Yukitoshi Katayama,
Sumie HiramatsuAsano,
Keiji Ohashi,
Michiko Morishita,
Mariko Narazaki,
Yoshinori Matsumoto,
Ken-Ei Sada,
Jun Wada
Publication year - 2021
Publication title -
medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.59
H-Index - 148
eISSN - 1536-5964
pISSN - 0025-7974
DOI - 10.1097/md.0000000000024028
Subject(s) - medicine , granulomatosis with polyangiitis , prednisolone , cyclophosphamide , vasculitis , pneumonia , respiratory tract , rituximab , gastroenterology , surgery , radiology , respiratory system , chemotherapy , disease , lymphoma
Rationale: Bronchial 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 concerns: A 65-year-old man complained of a 2-week cough and fever. Diagnoses: Considering 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. Interventions: He was treated with prednisolone (PSL, 50 mg/d) and intravenous cyclophosphamide. Outcomes: His 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. Lessons: GPA with bronchial involvement is often intractable and requires careful follow-up, which should include upper respiratory tract and hypertrophic pachymeningitis assessment.