小児多発血管炎性肉芽腫症例

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  • A Case of Granulomatosis with Polyangiitis in a Child
  • ショウニ タハツ ケッカンエンセイ ニクゲシュ ショウレイ

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<p>Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a rare anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. GPA in childhood is very rare, so that early diagnosis is difficult in pediatric patients. We report the case of a 14-year-old boy who presented with rapidly progressive disease. He was referred to our department with persistent severe right orbital pain and nasal obstruction despite receiving medication for a month. Right-sided sinusitis and bilateral orbital masses were confirmed on computed tomography (CT) and magnetic resonance imaging (MRI), and endoscopic sinus surgery was performed both the purpose of treating the sinusitis and performing biopsy. Histopathological examination revealed findings suggesting non-specific inflammation of the sinus, while the orbital pain and eyelid swelling continued to worsen, the sinusitis persisted, and the patient also complained of cough. As we suspected GPA, we performed biopsy of the right orbital mass and nasal septal mucosa, and also serology for PR-3 ANCA. As the histopathological results were typical of GPA and serology for PR-3 ANCA was positive, the patient was diagnosed as having GPA three weeks after his first visit to our hospital. The prognosis of GPA is very poor, with 90% of patients dying within a year if left untreated. As early diagnosis and start of treatment are crucial, the attending physician shohld have a high index of suspicion for GPA, even in pediatric patients.</p>

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