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特发性IgA系膜肾病。374例患者的临床与组织学研究

Idiopathic IgA mesangial nephropathy. Clinical and histological study of 374 patients.

作者信息

D'Amico G, Imbasciati E, Barbiano Di Belgioioso G, Bertoli S, Fogazzi G, Ferrario F, Fellin G, Ragni A, Colasanti G, Minetti L

出版信息

Medicine (Baltimore). 1985 Jan;64(1):49-60.

PMID:3880853
Abstract

Histological features and data on the natural history after 1 to 45 years (mean 6.56 +/- 8.55) of total apparent duration and 1 to 13 years (mean 3.48 +/- 5.04) of post-biopsy follow-up, are reported in 374 patients (mean age, 33.9 +/- 11.9 yrs) with idiopathic mesangial IgA nephropathy, who presented with a history of macroscopic hematuria (56%), recurrent in two-thirds of the patients, or with persistent microscopic hematuria and no previous episodes of gross hematuria (44%). Mesangial cell proliferation ranged from minimal to diffuse. Associated varying degrees of extracapillary proliferation, segmental and global glomerular sclerosis, tubulo-interstitial damage and arteriolar hyalinosis usually correlated with each other and with the extent of mesangial proliferation (P less than 0.05). The actuarial curve of progression to renal death showed a 75% survival after 20 years from apparent onset. Progression to renal failure was more rapid in patients with: an older age at onset (P = 0.0582); male sex (P = 0.0730); no history of recurrent gross hematuria (P = 0.0406); high blood pressure (P = 0.0011); more marked global (P = 0.0007) and segmental (P = 0.0026) glomerular sclerosis; more severe interstitial sclerosis (P = 0.0147); more diffuse and global mesangial proliferation (P = 0.0820); mesangio-parietal pattern at immunofluorescence (P = 0.0778). However, all these parameters showed a poor predictive value if applied to any single patient.

摘要

报告了374例(平均年龄33.9±11.9岁)特发性系膜IgA肾病患者的组织学特征以及总病程1至45年(平均6.56±8.55年)和活检后随访1至13年(平均3.48±5.04年)的自然史数据。这些患者有肉眼血尿病史(56%),其中三分之二患者血尿复发,或有持续性镜下血尿且既往无肉眼血尿发作史(44%)。系膜细胞增生程度从轻度到弥漫性不等。相关的不同程度的毛细血管外增生、节段性和全球性肾小球硬化、肾小管间质损伤和小动脉玻璃样变通常相互关联,且与系膜增生程度相关(P<0.05)。从明显发病起20年后的肾死亡精算曲线显示生存率为75%。在以下患者中进展至肾衰竭更快:发病年龄较大(P = 0.0582);男性(P = 0.0730);无肉眼血尿复发史(P = 0.0406);高血压(P = 0.0011);更明显的全球性(P = 0.0007)和节段性(P = 0.0026)肾小球硬化;更严重的间质硬化(P = 0.0147);更弥漫和全球性的系膜增生(P = 0.0820);免疫荧光显示系膜-壁层模式(P = 0.0778)。然而,如果将所有这些参数应用于任何单个患者,其预测价值都很差。

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