Am J Kidney Dis. 1985 Mar;5(3):157-64. doi: 10.1016/s0272-6386(85)80044-5.
In a multicenter study of 83 children with IgA nephropathy, we have encountered four patients (three female, one male) who presented with steroid-responsive nephrotic syndrome (NS) 1 to 12 years before the demonstration of IgA nephropathy. Renal biopsies performed within several months of the onset of NS in three of the patients showed changes consistent with minimal change glomerulonephropathy in two and diffuse mesangial hypercellularity in one patient. Immunofluorescence studies performed on two of the biopsies were negative for IgA. Corticosteroid therapy induced remission of NS in all four patients, but this was followed by frequent relapses. IgA nephropathy was diagnosed subsequently by renal biopsies that were performed because of the onset of gross hematuria in three patients and persistent, low-grade proteinuria in the other. Light microscopy studies on these biopsies showed minimal glomerular changes in three and focal proliferative and sclerosing glomerulonephritis in one patient. Subsequent follow-up evaluations have revealed normal serum creatinines and/or glomerular filtration rate (GFR) in all four patients with remission of NS in three and no further episodes of gross hematuria in any patient. These four patients represent either a variant of IgA nephropathy associated with long-standing steroid-responsive NS or development of IgA nephropathy superimposed upon preceding minimal change disease.
在一项针对83例IgA肾病患儿的多中心研究中，我们遇到了4例患者（3名女性，1名男性），他们在IgA肾病确诊前1至12年出现了激素反应性肾病综合征（NS）。其中3例患者在NS发病后数月内进行的肾活检显示，2例符合微小病变性肾小球肾炎的改变，1例表现为弥漫性系膜细胞增多。对其中2例活检组织进行的免疫荧光研究显示IgA阴性。4例患者的NS均通过皮质类固醇治疗诱导缓解，但随后频繁复发。随后，3例患者因肉眼血尿发作、另1例因持续性轻度蛋白尿而进行肾活检，从而确诊为IgA肾病。这些活检组织的光镜检查显示，3例肾小球改变轻微，1例为局灶增生性和硬化性肾小球肾炎。随后的随访评估显示，4例患者血清肌酐和/或肾小球滤过率（GFR）均正常，3例NS缓解，所有患者均未再出现肉眼血尿。这4例患者代表了与长期激素反应性NS相关的IgA肾病变体，或先前微小病变疾病基础上叠加的IgA肾病。
