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[免疫复合物新月体性肾小球肾炎伴MPO-ANCA持续高滴度6年病例]

[Case of immune complex crescentic glomerulonephritis with consistently high titers of MPO-ANCA for 6 years].

作者信息

Ayada Mikiko, Matsuo Takatoshi, Takada Shigeru, Kusaura Takashi, Suda Shin, Okado Tomokazu, Mori Yoshihiro, Tajima Masato, Kuwahara Michio, Kobayashi Yutaka, Tsukamoto Yusuke, Sasaki Sei

机构信息

Division of Nephrology, Shuwa General Hospital, Saitama, Japan.

出版信息

Nihon Jinzo Gakkai Shi. 2007;49(5):511-6.

Abstract

A male patient, now 65 years old, experienced fever, hemoptysis, and respiratory failure about six years ago. Soon thereafter, he developed rapid progressive renal dysfunction with pulmonary hemorrhage and positive findings for MPO-ANCA. We commenced methylprednisolone pulse (MP) therapy followed by oral prednisolone (PSL) and intravenous cyclophosphamide (CY) for the treatment of ANCA-associated microscopic polyangiitis (MPA). Therapeutic efficacy was obtained comparatively rapidly. Light microscopic findings of a percutaneous renal biopsy demonstrated focal necrotizing and crescentic glomerulonephritis. Immunofluorescent microscopy indicated diffuse deposition of IgG and C3 along the periphery of the tufts and in the mesangium. On the basis of these findings, the condition was diagnosed as immune complex crescentic glomerulonephritis associated with MPO-ANCA. MPO-ANCA titers were high (714 EU) at onset and remained high (250-450 EU) over the ensuing 6 years with oral administration of PSL 5 mg. Though his condition remitted completely, his MPO-ANCA titers recently increased to above 600 EU once more. We conducted a follow-up renal biopsy to ascertain if the fluctuation of MPO-ANCA titers reflected an early stage of relapse. Light microscopic findings of the biopsied tissue revealed no signs of necrosis or crescentic formation of the glomeruli. Immunofluorescent microscopic findings were negative. The elevated MPO-ANCA titers were not valuable for the early prediction of relapse in our case, and the immune complex may have played an important role. When judging relapse and remission in ANCA-associated glomerulonephritis, it is important to evaluate the overall clinical findings and histopathological findings in addition to the serial ANCA titers.

摘要

一名65岁男性患者大约6年前出现发热、咯血和呼吸衰竭。此后不久,他发展为快速进行性肾功能不全,伴有肺出血,MPO-ANCA检测呈阳性。我们开始采用甲泼尼龙冲击(MP)治疗,随后口服泼尼松龙(PSL)和静脉注射环磷酰胺(CY)来治疗抗中性粒细胞胞浆抗体相关性显微镜下多血管炎(MPA)。治疗效果相对较快显现。经皮肾活检的光镜检查显示局灶性坏死性和新月体性肾小球肾炎。免疫荧光显微镜检查表明IgG和C3沿肾小球毛细血管袢外周和系膜区弥漫性沉积。基于这些发现,该疾病被诊断为与MPO-ANCA相关的免疫复合物新月体性肾小球肾炎。发病时MPO-ANCA滴度较高(714 EU),在随后6年口服5 mg PSL期间一直维持在较高水平(250 - 4�0 EU)。尽管他的病情完全缓解,但最近他的MPO-ANCA滴度再次升至600 EU以上。我们进行了一次随访肾活检,以确定MPO-ANCA滴度的波动是否反映了复发的早期阶段。活检组织的光镜检查未发现肾小球坏死或新月体形成的迹象。免疫荧光显微镜检查结果为阴性。在我们的病例中,MPO-ANCA滴度升高对复发的早期预测并无价值,免疫复合物可能起到了重要作用。在判断抗中性粒细胞胞浆抗体相关性肾小球肾炎的复发和缓解时,除了连续监测抗中性粒细胞胞浆抗体滴度外,评估整体临床症状和组织病理学表现也很重要。

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