Department of Rheumatology, Kameda Medical Center, Kamogawa, Chiba, Japan.
Department of Nephrology, Kameda Medical Center, 929 Higashi-cho, Kamogawa, Chiba, Japan.
CEN Case Rep. 2023 Feb;12(1):50-55. doi: 10.1007/s13730-022-00719-w. Epub 2022 Jul 11.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a systemic inflammation of small or medium blood vessels that includes microscopic polyangiitis. A diagnosis of ANCA-associated vasculitis can be aided by histological identification of vasculitis, and identification of renal impairment can help predict outcomes. However, kidney biopsy is not generally indicated in the absence of renal findings. We report two cases of ANCA-associated vasculitis diagnosed by kidney biopsy despite the absence of remarkable urinary abnormality and renal impairment. These patients had fever of unknown origin and were positive for myeloperoxidase (MPO)-ANCA but showed few findings that would suggest small-vessel vasculitis in the kidney. Nevertheless, kidney biopsies revealed small-vessel arteritis, necrotizing glomerulonephritis, and interstitial nephritis. Immunofluorescent antibody tests performed using samples of glomeruli were all negative, suggesting microscopic polyangiitis. Therefore, kidney biopsy may be useful in confirming the diagnosis, even if patients have completely normal urinary findings in the absence of other organ lesions.
抗中性粒细胞胞浆抗体(ANCA)相关性血管炎是一种小或中等血管的系统性炎症,包括显微镜下多血管炎。组织学识别血管炎和确定肾损害有助于诊断 ANCA 相关性血管炎,并且有助于预测预后。然而,在没有肾脏发现的情况下,通常不建议进行肾活检。我们报告了两例肾活检诊断为 ANCA 相关性血管炎的病例,尽管患者无明显的尿异常和肾功能损害。这些患者有不明原因的发热,且髓过氧化物酶(MPO)-ANCA 阳性,但肾脏表现出的小血管血管炎的发现很少。然而,肾活检显示小血管炎、坏死性肾小球肾炎和间质性肾炎。使用肾小球样本进行的免疫荧光抗体试验均为阴性,提示显微镜下多血管炎。因此,即使患者在没有其他器官病变的情况下完全正常的尿液发现,肾活检也可能有助于确诊。