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Granulomatosis with polyangiitis with obstructive pneumonia progressing to hypertrophic pachymeningitis: A case

Keigo Hayashi¹, Haruki Watanabe, Yuriko Yamamura

¹Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.

|February 6, 2021

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.

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