Iijima Takashi, Suwabe Tatsuya, Sumida Keiichi, Hayami Noriko, Hiramatsu Rikako, Hasegawa Eiko, Yamanouchi Masayuki, Hoshino Junichi, Sawa Naoki, Takaichi Kenmei, Oohashi Kenichi, Fujii Takeshi, Ubara Yoshifumi
Nephrology Center, Toranomon Hospital Kajigaya , 1-3-1 Kajigaya, Takatsu, 213-8587, Kawasaki, Kanagawa , Japan.
Mod Rheumatol. 2015 Jan;25(1):138-42. doi: 10.3109/14397595.2013.874748. Epub 2014 Feb 18.
We report a Japanese woman with systemic rheumatoid vasculitis (SRV) complicated by necrotizing crescentic glomerulonephritis (NCGN). Rheumatoid arthritis first occurred at the age of 19 years, followed by interstitial pneumonia, hepatitis, rheumatoid nodules, mononeuritis multiplex, and hypocomplementemia in chronological order. At the age of 51 years, rapidly progressive renal failure occurred with nephrotic proteinuria, and NCGN with subepithelial deposits was revealed by renal biopsy. Severe destructive changes of multiple joints and scleritis were detected, but anti-neutrophil cytoplasmic antibody was negative on enzyme-linked immunosorbent assays and indirect immunofluorescence. SRV was diagnosed due to involvement of multiple extra-articular organs. An anti-interleukin (IL)-6 receptor antibody (tocilizumab) was started at dosage of 280 mg (8 mg/kg) monthly. After 18 months, her serum creatinine decreased from 1.7 to 1.3 mg/dL, and urinary protein excretion declined from 5.2 to 1.2 g daily. Tocilizumab may be a therapeutic option for SRV associated with NCGN.
我们报告了一名患有系统性类风湿性血管炎（SRV）并伴有坏死性新月体性肾小球肾炎（NCGN）的日本女性。类风湿性关节炎首次发病于19岁，随后依次出现间质性肺炎、肝炎、类风湿结节、多发性单神经炎和补体血症降低。51岁时，出现快速进展性肾衰竭并伴有肾病性蛋白尿，肾活检显示为伴有上皮下沉积物的NCGN。检测到多个关节出现严重破坏性改变和巩膜炎，但酶联免疫吸附测定和间接免疫荧光法检测抗中性粒细胞胞浆抗体为阴性。由于多个关节外器官受累而诊断为SRV。开始每月以280mg（8mg/kg）的剂量使用抗白细胞介素（IL）-6受体抗体（托珠单抗）。18个月后，她的血清肌酐从1.7mg/dL降至1.3mg/dL，尿蛋白排泄量从每日5.2g降至1.2g。托珠单抗可能是与NCGN相关的SRV的一种治疗选择。
