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

IF 0.9 Q4 RHEUMATOLOGY
Takumi Saito, Wataru Nakamura, Yujin Nishioka, Erika Matsuda, Mariko Yamana, Rina Takahashi, Masahiro Kogami, Ayako Makiyama, Goh Murayama, Yoshiyuki Abe, Takuo Hayashi, Makio Kusaoi, Kurisu Tada, Ken Yamaji, Naoto Tamura
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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.

肉芽肿性鼻窦炎颅内侵犯:一例罕见的肉芽肿病合并多血管炎。
肉芽肿病合并多血管炎(GPA)累及中枢神经系统并不常见,颅内肿块病变的形成尤其罕见。我们描述的情况下,日本妇女在她的三十多岁与GPA最初仅限于上呼吸道。发病12年后,脑MRI显示额叶有分叶状肿块,CT显示肉芽肿性炎症从鼻窦直接延伸至筛网板。由于感染的风险与筛状板破坏相关,手术切除是为了诊断和预防目的。切除的颅内病变的组织病理学检查显示坏死肉芽肿没有感染的证据,与GPA一致。术后,给予中等剂量的强的松龙和利妥昔单抗,导致临床和血清学缓解。本病例强调了一种罕见的由鼻窦直接连续扩散引起的GPA颅内表现,这种情况可能在没有全身症状的情况下发生。尽管鼻窦受累通常被认为不严重,但骨破坏的存在可能预示着潜在的器官威胁过程。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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CiteScore
1.40
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