Departamento de Patología, Facultad de Medicina, Universidad de Antioquia y Hospital Universitario San Vicente de Paúl, Medellín, Antioquia, Colombia.
Nefrologia. 2013;33(4):532-8. doi: 10.3265/Nefrologia.pre2013.Mar.11962.
IgM nephropathy (IgMN) is a glomerulonephritis characterised by diffuse mesangial immunoglobulin M (IgM) deposits. It usually presents with nephrotic range proteinuria and, according to some previous work, it occurs most often in patients who are resistant to or dependent on steroid treatment.
To perform a clinical, histological and immunopathological description and assess the response to steroid treatment of paediatric patients diagnosed with nephrotic syndrome and diffuse mesangial IgM deposits.
This is a descriptive, retrospective study carried out in two hospitals, where the clinical records of paediatric patients with IgMN were analysed and the histological sections were re-assessed.
thirteen children were included in this study. IgMN corresponded to 5.17% of all paediatric renal biopsies. The age of patients ranged from 1 year to 12 years (median: 2 years), 46.7% were women. The most common morphological finding was diffuse mesangial hypercellularity (46.1%), followed by focal segmental glomerulosclerosis (30.8%) and minimal glomerular changes (23.1%). All patients received steroids; in 4 cases (30.7%) as the only immunosuppressant medication, 3 (23.1%) also received cyclophosphamide, 5 (38.4%) mycophenolate, and 1 (7.7%) cyclosporine. Seven patients (53.8%) had frequent relapses, 5 (38.5%) were cortico-resistant and 1 (7.7%) cortico-dependent. Two patients (15.38%) had chronic impairment of renal function.
The presence of diffuse mesangial IgM in paediatric patients with nephrotic syndrome is not a very uncommon finding; its clinical presentation has been associated with lower response to steroids. However, the long-term prognosis of these patients is still unknown.
IgM 肾病（IgMN）是一种肾小球肾炎，其特征为弥漫性系膜免疫球蛋白 M（IgM）沉积。它通常表现为肾病范围内的蛋白尿，根据一些先前的研究，它最常发生在对类固醇治疗有抵抗力或依赖的患者中。
对诊断为肾病综合征和弥漫性系膜 IgM 沉积的儿科患者进行临床、组织学和免疫病理学描述，并评估其对类固醇治疗的反应。
这是一项在两家医院进行的描述性、回顾性研究，对 IgMN 儿科患者的临床记录进行分析，并重新评估组织学切片。
本研究共纳入 13 例儿童。IgMN 占所有儿科肾活检的 5.17%。患者年龄从 1 岁到 12 岁不等（中位数：2 岁），46.7%为女性。最常见的形态学发现是弥漫性系膜细胞增生（46.1%），其次是局灶节段性肾小球硬化（30.8%）和微小肾小球病变（23.1%）。所有患者均接受类固醇治疗；在 4 例（30.7%）中作为唯一的免疫抑制剂药物，3 例（23.1%）还接受环磷酰胺治疗，5 例（38.4%）接受霉酚酸酯治疗，1 例（7.7%）接受环孢素治疗。7 例（53.8%）患者频繁复发，5 例（38.5%）患者对皮质类固醇耐药，1 例（7.7%）患者对皮质类固醇依赖。2 例（15.38%）患者出现肾功能慢性损害。
在肾病综合征的儿科患者中，弥漫性系膜 IgM 的存在并不罕见；其临床表现与对类固醇的反应较低有关。然而，这些患者的长期预后仍不清楚。
Zotero 插件