Niimi Naoko, Miyashita Tomoko, Tanji Kana, Hirai Takuya, Watanabe Kozo, Ikeda Keigo, Morimoto Shinji, Sekigawa Iwao
Department of Anesthesia, Juntendo University Urayasu Hospital, 2-1-1 Tomioka, Urayasu, Chiba 279-0021, Japan.
Department of Internal Medicine and Rheumatology, School of Medicine, Juntendo University, 2-1-1 Hongo, Bunkyo, Tokyo 113-8421, Japan.
Case Rep Rheumatol. 2018 Jun 5;2018:9682801. doi: 10.1155/2018/9682801. eCollection 2018.
A 57-year-old Japanese man was admitted to the hospital with back pain and fever, multiple lung nodules, and abdominal aortic aneurysm (AAA). Laboratory tests performed at admission showed an increased proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA) level. Video-associated thoracoscopic lung biopsy was performed; pathologic examination showed granulation tissue with necrosis and multinucleated giant cells. The diagnosis of granulomatosis with polyangiitis (GPA) was confirmed on the basis of the clinical presentation, laboratory findings, and lung biopsy. All symptoms were ameliorated, and the serum level of PR3-ANCA declined following treatment with prednisolone and cyclophosphamide. Although the association of GPA with AAA is rare, GPA may be included among the large vessel vasculitides that can give rise to aortic aneurysm.
一名57岁的日本男性因背痛、发热、多发肺结节和腹主动脉瘤(AAA)入院。入院时进行的实验室检查显示蛋白酶3抗中性粒细胞胞浆抗体(PR3-ANCA)水平升高。进行了电视辅助胸腔镜肺活检;病理检查显示有坏死和多核巨细胞的肉芽组织。根据临床表现、实验室检查结果和肺活检,确诊为肉芽肿性多血管炎(GPA)。使用泼尼松龙和环磷酰胺治疗后,所有症状均得到改善,PR3-ANCA血清水平下降。虽然GPA与AAA的关联罕见,但GPA可能属于可导致主动脉瘤的大血管血管炎。