外科的肺生検で診断された抗好中球細胞質抗体陰性肺限局型多発血管炎性肉芽腫症

Antineutrophil Cytoplasmic Antibody （ANCA） Negative Limited-form Granulomatosis with Polyangiitis of the Lung Diagnosed by the Thoracoscopic Lung Biopsy

多発血管炎性肉芽腫症（GPA）は，従来Wege‑ner肉芽腫症とされていた疾患で，上下気道や腎病変を有する全身型の場合は抗好中球細胞質抗体（ANCA）陽性を示し，比較的容易に診断可能であるが，ANCA陰性限局型の診断は困難な場合が多く，病理組織診断が決め手となる．われわれは胸部X線像で6年の経過中に消長を繰り返した結節陰影について，最終的に胸腔鏡下肺生検病理像から肺限局型ANCA陰性GPAと診断しえた1例を経験した．1995年以降の本邦報告7例の解析も含めて報告する．

Antineutrophil cytoplasmic antibody （ANCA） negative pulmonary limited-form granulomatous with polyangiitis （GPA） is a rare type of GPA. A 53-year-old female had been followed as the possible pulmonary infarction of bilateral lungs for 4 years without any therapy. Chest computed tomography （CT） examination of the patient showed newly appeared nodular lesions in the lungs, which were suspected as malignancy by positron emission tomography （PET） -CT. Thoracoscopic lung biopsy of the lesions was performed and histopathological diagnosis was GPA showing multiple granulomas with vasculitis. Since both C and P-ANCA were negative and no evidence of kidney involvement, we finally diagnosed the lung lesions as ANCA negative limited-form GPA.