Intracranial invasion of granulomatous sinusitis: report of a rare case of granulomatosis with polyangiitis

The involvement of the central nervous system with granulomatosis with polyangiitis (GPA) is uncommon, and the formation of intracranial mass lesions is particularly rare. We describe the case of a Japanese woman in her thirties with GPA initially limited to the upper respiratory tract. Twelve years after the disease was onset, brain magnetic resonance imaging revealed a lobulated mass in the frontal lobe, and computed tomography findings suggested direct extension of granulomatous inflammation from the paranasal sinuses through the cribriform plate. Due to the risk of infection associated with cribriform plate destruction, surgical resection was performed for both diagnostic and preventive purposes. Histopathological examination of the resected intracranial lesion revealed necrotising granulomas without evidence of infection, consistent with GPA. Postoperatively, a moderate dose of prednisolone and rituximab was administered, resulting in clinical and serological remission. This case highlights a rare intracranial manifestation of GPA caused by direct contiguous spread from the paranasal sinuses, which can occur in the absence of systemic symptoms. Although sinonasal involvement is typically regarded as non-severe, the presence of bony destruction may signal a potentially organ-threatening course.

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Publication Details

Journal
Modern Rheumatology Case Reports
Published
2025-01-01
DOI
https://doi.org/10.1093/mrcr/rxaf058
Primary Topic
Vasculitis and related conditions
Type
article
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article

Intracranial invasion of granulomatous sinusitis: report of a rare case of granulomatosis with polyangiitis

Ayako Makiyama, Goh Murayama, Kurisu Tada, Makio Kusaoi et al.
Modern Rheumatology Case Reports
Vasculitis and related conditions
article

Intracranial invasion of granulomatous sinusitis: report of a rare case of granulomatosis with polyangiitis

Ayako Makiyama, Goh Murayama, Kurisu Tada, Makio Kusaoi, Takuo Hayashi, Rina Takahashi, Naoto Tamura, Masaki Yamana, Masahiro Kogami, Yoshiyuki Abe, Wataru Nakamura, Yujin Nishioka, Taís Berelli Saito, Ken Yamaji, Eriko Matsuda
article en

Abstract

The involvement of the central nervous system with granulomatosis with polyangiitis (GPA) is uncommon, and the formation of intracranial mass lesions is particularly rare. We describe the case of a Japanese woman in her thirties with GPA initially limited to the upper respiratory tract. Twelve years after the disease was onset, brain magnetic resonance imaging revealed a lobulated mass in the frontal lobe, and computed tomography findings suggested direct extension of granulomatous inflammation from the paranasal sinuses through the cribriform plate. Due to the risk of infection associated with cribriform plate destruction, surgical resection was performed for both diagnostic and preventive purposes. Histopathological examination of the resected intracranial lesion revealed necrotising granulomas without evidence of infection, consistent with GPA. Postoperatively, a moderate dose of prednisolone and rituximab was administered, resulting in clinical and serological remission. This case highlights a rare intracranial manifestation of GPA caused by direct contiguous spread from the paranasal sinuses, which can occur in the absence of systemic symptoms. Although sinonasal involvement is typically regarded as non-severe, the presence of bony destruction may signal a potentially organ-threatening course.

Modern Rheumatology Case ReportsVol. 9(2)
Juntendo University (JP)
Openalex Percentile: Top 63%
Vasculitis and related conditions
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