Granulomatosis with polyangiitis with obstructive pneumonia progressing to hypertrophic pachymeningitis: A case report. Issue 3 (22nd January 2021)

• Record Type: Journal Article
• Authors: Hayashi, Keigo; Watanabe, Haruki; Yamamura, Yuriko; Asano, Yosuke; Katayama, Yu; Hiramatsu-Asano, Sumie; Ohashi, Keiji; Morishita, Michiko; Narazaki, Mariko; Matsumoto, Yoshinori; Sada, Ken-Ei; Wada, Jun
• Other Names: Saranathan. Maya section editor.
• Is Part Of: Medicine. Volume 100:Issue 3(2021)
• Publication Date: 2021-01-22
• Subjects: bronchial stenosis -- granulomatosis with polyangiitis -- hypertrophic pachymeningitis -- rituximab; Medicine -- Periodicals; Medicine -- Periodicals; Médecine -- Périodiques; Geneeskunde; Medicine; Periodicals; Periodicals; 610.5
• DOI: 10.1097/MD.0000000000024028
• Languages: English
• ISSNs: 0025-7974

Abstract

Abstract: 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.