D'Amico G, Imbasciati E, Barbiano Di Belgioioso G, Bertoli S, Fogazzi G, Ferrario F, Fellin G, Ragni A, Colasanti G, Minetti L
Medicine (Baltimore). 1985 Jan;64(1):49-60.
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.
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。
