Hatanaka Saeko, Kanzaki Go, Koike Kentaro, Ueda Hiroyuki, Matsuo Nanae, Maruyama Yukio, Tsuboi Nobuo, Yokoo Takashi
Division of Nephrology and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, Minato-Ku, Tokyo, 105-8461, Japan.
CEN Case Rep. 2025 Jun;14(3):486-492. doi: 10.1007/s13730-024-00961-4. Epub 2024 Dec 28.
Immunoglobulin A nephropathy (IgAN) is the most common primary glomerulonephritis worldwide with heterogeneous histopathological phenotypes. Although IgAN with membranoproliferative glomerulonephritis (MPGN)-like features has been reported in children and adults, treatment strategies for this rare IgAN subtype have not been established. Here, we present the case of a 56-year-old man with no history of kidney disease who initially presented with nephrotic syndrome. Renal biopsy revealed MPGN-like features with a negative serological workup for secondary causes. Immunofluorescent staining was predominantly positive for IgA in the glomerular mesangial and capillary walls. Galactose-deficient IgA1 staining showed a distribution pattern similar to IgA staining. Electron microscopy revealed disorganized structural deposits in the mesangial and subendothelial regions. Based on clinical and histopathological findings, the patient was diagnosed with primary IgAN. The nephrotic syndrome resolved completely after six months of combined corticosteroids and cyclosporine A (CsA) therapy. Although corticosteroids and CsA were tapered off, hematuria and proteinuria remained in complete remission for years of follow-up. This case demonstrates the importance of recognizing IgAN with MPGN-like features as a histopathological subtype that may benefit from intensive immunosuppressive therapy.
免疫球蛋白A肾病(IgAN)是全球最常见的原发性肾小球肾炎,具有异质性组织病理学表型。尽管儿童和成人中均有IgAN伴膜增生性肾小球肾炎(MPGN)样特征的报道,但这种罕见的IgAN亚型的治疗策略尚未确立。在此,我们报告一例56岁无肾脏疾病史的男性患者,其最初表现为肾病综合征。肾活检显示具有MPGN样特征,继发性病因的血清学检查结果为阴性。免疫荧光染色显示肾小球系膜和毛细血管壁IgA主要呈阳性。缺乏半乳糖的IgA1染色显示出与IgA染色相似的分布模式。电子显微镜检查显示系膜和内皮下区域存在结构紊乱的沉积物。根据临床和组织病理学检查结果,该患者被诊断为原发性IgAN。联合使用皮质类固醇和环孢素A(CsA)治疗6个月后,肾病综合征完全缓解。尽管逐渐减少了皮质类固醇和CsA的用量,但在多年随访中血尿和蛋白尿仍完全缓解。该病例表明,认识到具有MPGN样特征的IgAN作为一种组织病理学亚型的重要性,其可能从强化免疫抑制治疗中获益。