浸潤型真菌症や悪性腫瘍との鑑別を要した上顎洞原発多発血管炎性肉芽腫症症例

A case of granulomatosis with polyangiitis of the maxillary sinus requiring differentiation from invasive fungal infection and malignant tumor

はじめに

　多発血管炎性肉芽腫症（granulomatosis with polyangiitis：GPA）は，以前はWegener肉芽腫症と呼ばれており，Wegener^1）が1936年に上・下気道の壊死性肉芽腫性病変・全身の血管炎・壊死性腎炎を3徴とする原因不明の全身性疾患を報告したことから始まっている。初発症状として鼻症状を呈する者は多く，鼻腔内痂皮形成・鼻中隔穿孔・鞍鼻などを認めた場合は本疾患を鑑別に挙げるべきであることはすでによく知られているが，鼻腔所見に明らかな病変がない副鼻腔原発の症例報告は少ない^2）。

　今回われわれは，上顎洞周囲の骨破壊を伴い，浸潤型真菌症や悪性腫瘍との鑑別を要したGPAの症例を経験したので報告する。

　Granulomatosis with polyangiitis（GPA）often develops with nasal symptoms. GPA without nasal symptoms is difficult to diagnose. We experienced a case of granulomatosis with polyangiitis of the maxillary sinus requiring differentiation from invasive fungal infection and malignant tumor.

　A 71-year-old woman presented with left cheek pain. CT tomography revealed a soft tissue shadow accompanied by bone destruction of the maxillary sinus, although her nasal findings were normal. Invasive fungal infections and malignant tumors were preoperatively suspected. Endoscopic sinus surgery was performed, and GPA was diagnosed. If we focused on preoperative lung lesions, we may have been able to make an accurate preoperative diagnosis. She was treated with Rituximab and Methylprednisolone at the Department of Internal Medicine of our hospital. She has been in remission for approximately 6 months since the surgery.