Asakawa Tomohiko, Asou Mea, Hara Shigeo, Ehara Takashi, Araki Makoto
Department of Internal Medicine, Suwa Central Hospital, Chino, Nagano 391-8503, Japan.
Department of Diagnostic Pathology, Kobe City Medical Center General Hospital, 2-1-1, Minatojima Minamimachi, Chuo-ku, Kobe 650-0047, Japan.
Case Rep Nephrol. 2020 Mar 23;2020:6343521. doi: 10.1155/2020/6343521. eCollection 2020.
An elderly woman was admitted with the chief complaint of gross hematuria. Laboratory values indicated a high myeloperoxidase-ANCA level. In renal histological examination, 40% of the glomeruli showed crescent formation, but immunofluorescence staining showed positivity for IgG, C3, and C1q. Furthermore, the deposition of fibrils in the glomerulus was noted on electron microscopy, and immunohistochemical staining showed strong positivity for DNA-J heat shock protein family member B9 (DNAJB9). Crescent formation is a common feature of fibrillary glomerulonephritis (FGN). Thus, in ANCA-positive crescentic glomerulonephritis, immunohistochemical assessments for immunoglobulins and DNAJB9, as well as electron microscopy, are important to correctly diagnose FGN.
一名老年女性因肉眼血尿为主诉入院。实验室检查结果显示髓过氧化物酶-抗中性粒细胞胞浆抗体（MPO-ANCA）水平升高。肾脏组织学检查显示，40%的肾小球有新月体形成，但免疫荧光染色显示IgG、C3和C1q呈阳性。此外，电子显微镜下可见肾小球中有纤维沉积，免疫组织化学染色显示DNA-J热休克蛋白家族成员B9（DNAJB9）呈强阳性。新月体形成是纤维性肾小球肾炎（FGN）的常见特征。因此，在ANCA阳性的新月体性肾小球肾炎中，免疫球蛋白和DNAJB9的免疫组织化学评估以及电子显微镜检查对于正确诊断FGN很重要。
