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科威特与显微镜下多血管炎和经典结节性多动脉炎相关的肾脏疾病谱。

The spectrum of renal disease associated with microscopic polyangiitis and classic polyarteritis nodosa in Kuwait.

作者信息

el-Reshaid K, Kapoor M M, el-Reshaid W, Madda J P, Varro J

机构信息

Department of Medicine, Faculty of Medicine, Kuwait University, Kuwait.

出版信息

Nephrol Dial Transplant. 1997 Sep;12(9):1874-82. doi: 10.1093/ndt/12.9.1874.

DOI:10.1093/ndt/12.9.1874
PMID:9306338
Abstract

BACKGROUND

This study was conducted to examine prospectively the epidemiological profile of renal disease associated with microscopic polyangiitis (MPA) and classic polyarteritis nodosa (classic PAN) in Kuwait and its clinicopathological correlation.

METHODS

Between 1 January 1993 and 31 December 1996, all patients referred to Al-Amiri renal centre were subjected to renal biopsy and/or arteriography if they manifested systemic manifestations of vasculitis or had rapid or unexplained renal failure with or without proteinuria. Patients who fulfilled the criteria of MPA and classic PAN, according to the Chapel Hill Consensus Conference (CHCC), were included in the study.

RESULTS

Over those 4 years, 47 patients were diagnosed as having such vasculitides, 22 of whom were Kuwaiti nationals. This gave an average annual incidence of 45 cases/million adult Kuwaiti nationals (95% CI, 27-64). Histological examination revealed necrotizing glomerulonephritis (GN) in 11 patients and crescentic GN in 13, while isolated small- and/or medium-sized-arteritis were evident in six more patients. In 10 of the remaining patients; bands of fibrosis alternating with healthy renal tissue were seen and on arteriography non-atherosclerotic stenoses, infarctions, and/or microaneurysms were evident. The remaining seven patients were diagnosed by arteriography alone, five of whom had bilateral small kidneys. Constitutional manifestations of vasculitis were present in 32 patients (68%) and multisystem involvement in 19 (40%). ANCA levels were high in 24 (55%) of the 44 patients tested and seropositivity, as an antimyeloperoxidase, was a constant finding. Only six patients presented with rapidly progressive renal failure, while 31 presented with chronic renal disease, 18 of whom had protein excretion in excess of 2 g/day.

CONCLUSIONS

These findings emphasize the limited role of clinical assessment in establishing a definite diagnosis of renal vasculitis and put in question its value in retrospective analysis of causes of ESRD in different populations.

摘要

背景

本研究旨在前瞻性地调查科威特与显微镜下多血管炎(MPA)和经典结节性多动脉炎(经典PAN)相关的肾脏疾病的流行病学特征及其临床病理相关性。

方法

在1993年1月1日至1996年12月31日期间,所有转诊至阿米里肾脏中心的患者,若出现血管炎的全身表现,或伴有或不伴有蛋白尿的快速或不明原因的肾衰竭,均接受肾脏活检和/或血管造影检查。根据查珀尔希尔共识会议(CHCC)标准符合MPA和经典PAN标准的患者纳入本研究。

结果

在这4年中,47例患者被诊断患有此类血管炎,其中22例为科威特国民。这使得成年科威特国民的年平均发病率为45例/百万(95%可信区间,27 - 64)。组织学检查显示,11例患者有坏死性肾小球肾炎(GN),13例有新月体性GN,另有6例患者有孤立的小和/或中动脉炎。在其余10例患者中,可见纤维化带与健康肾组织交替,血管造影显示非动脉粥样硬化性狭窄、梗死和/或微动脉瘤。其余7例患者仅通过血管造影诊断,其中5例双侧肾脏较小。32例患者(68%)有血管炎的全身表现,19例(40%)有多系统受累。在44例接受检测的患者中,24例(55%)抗中性粒细胞胞浆抗体(ANCA)水平较高,作为抗髓过氧化物酶的血清阳性是一个持续发现。只有6例患者表现为快速进行性肾衰竭,而31例表现为慢性肾病,其中18例每日蛋白尿排泄超过2g。

结论

这些发现强调了临床评估在明确诊断肾血管炎中的有限作用,并对其在不同人群中回顾性分析终末期肾病病因的价值提出质疑。

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