Oshima Yoichi, Suwabe Tatsuya, Marui Yuji, Hayami Noriko, Hasegawa Eiko, Yamanouchi Masayuki, Hiramatsu Rikako, Sumida Keiichi, Kawada Masahiro, Sekine Akinari, Mizuno Hiroki, Oguro Masahiko, Hoshino Junichi, Sawa Naoki, Ishii Yasuo, Fujii Takeshi, Ohashi Kenichi, Takaichi Kenmei, Ubara Yoshifumi
Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato, Tokyo, 105-8470, Japan.
Department of Pathology, Toranomon Hospital, Tokyo, Japan.
CEN Case Rep. 2018 Nov;7(2):274-281. doi: 10.1007/s13730-018-0342-1. Epub 2018 Jun 5.
A 29-year-old woman with past medical history of hypertension was referred to our hospital for the evaluation of kidney dysfunction (serum creatinine 1.0 mg/dL), proteinuria (0.54 g/gCre), and microscopic hematuria. Renal biopsy before the first pregnancy was supportive for benign nephrosclerosis with no evidence of vasculitis. After her second pregnancy and delivery when she was 32 years old, she developed proteinuria of 3.2 g/gCre, hematuria, and elevated serum creatinine level of 2.6 mg/dL. Second renal biopsy revealed necrotizing glomerulonephritis and her serum MPO-ANCA was positive, leading to the diagnosis of MPA/renal-limited vasculitis (RLV). Interestingly, frozen preserved serum from 4 years earlier also tested positive for MPO-ANCA. Despite intensive treatment, hemodialysis was required 10 years later due to progressive deterioration of renal function. At that time, she developed pericarditis, bloody cardiac tamponade, and pulmonary alveolar hemorrhage, resulting in a diagnosis of systemic vasculitis MPA. She received living donor kidney transplantation at the age of 44 years, after which serum creatinine has been stable around 1.1 mg/dL without proteinuria or hematuria and MPO-ANCA has remained negative. The association of vasculitis with pregnancy and delivery is not well documented, especially in patients with MPA. Here, we report this MPO-ANCA positive woman developing MPA necrotizing glomerulonephritis after her second pregnancy and a 20-year clinical course.
一名有高血压病史的29岁女性因肾功能不全（血清肌酐1.0mg/dL）、蛋白尿（0.54g/gCre）和镜下血尿被转诊至我院。首次怀孕前的肾活检支持良性肾硬化，无血管炎证据。她32岁第二次怀孕并分娩后，出现蛋白尿3.2g/gCre、血尿，血清肌酐水平升高至2.6mg/dL。第二次肾活检显示坏死性肾小球肾炎，其血清MPO-ANCA阳性，诊断为显微镜下多血管炎/肾局限性血管炎（RLV）。有趣的是，4年前冷冻保存的血清MPO-ANCA检测也呈阳性。尽管进行了强化治疗，但10年后由于肾功能进行性恶化仍需要血液透析。当时，她出现了心包炎、血性心脏压塞和肺泡出血，诊断为系统性血管炎显微镜下多血管炎。她44岁时接受了活体供肾移植，此后血清肌酐稳定在1.1mg/dL左右，无蛋白尿或血尿，MPO-ANCA仍为阴性。血管炎与妊娠和分娩的关联记录不详，尤其是在显微镜下多血管炎患者中。在此，我们报告这名MPO-ANCA阳性女性在第二次怀孕后发生显微镜下多血管炎坏死性肾小球肾炎及20年的临床病程。
