Raj Rahul, Sharma Alok, Barwad Adarsh, Bagchi Soumita, Agarwal Sanjay Kumar, Bagga Arvind, Dinda Amit Kumar, Singh Geetika
Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.
Department of Renal Pathology and Electron Microscopy, National Reference Lab, Lal Path Labs, New Delhi, India.
Glomerular Dis. 2021 Nov 2;2(2):59-74. doi: 10.1159/000520640. eCollection 2022 Apr.
Mucosal-derived galactose-deficient IgA is central to the pathogenesis of primary IgA nephropathy (IgAN). Recent reports suggest similar pathogenesis in Henoch-Schonlein purpura (HSP) and secondary IgAN. Its role in other IgA-containing glomerular diseases is still under investigation. It can be detected in glomeruli with the recently described antibody KM55. We aimed to evaluate the role of KM55 by immunostaining a wide spectrum of IgA-containing glomerular diseases.
After standardization and colocalization in a case of IgAN, a spectrum of 60 cases including IgAN, HSP, chronic liver disease (CLD)-related IgAN, other secondary IgAN, IgA-dominant/codominant membranoproliferative glomerulonephritis (MPGN), and lupus nephritis were subjected to immunofluorescence with KM55. KM55 was used to resolve diagnostic dilemma in cases of IgA deposition with confounding histology.
The group of primary IgAN (17 cases), HSP (4 cases), and secondary IgAN (19 cases) including CLD showed 2-3+ granular staining with KM55, suggesting mucosal-derived IgA. In contrast, cases of IgA-dominant/codominant MPGN (8 cases) and lupus nephritis (12 cases) were negative for KM55, suggesting systemic derivation of IgA. In cases of IgA deposition with confounding histology such as membranoproliferative or diffuse endocapillary proliferative pattern, KM55 helped to resolve the diagnosis.
DISCUSSION/CONCLUSION: This cross-sectional study concludes that KM55 is useful in the evaluation of IgA-containing glomerular diseases from a pathogenetic perspective and is a practical tool in resolving differential diagnosis in cases with overlapping histopathological features.
黏膜来源的半乳糖缺乏型IgA是原发性IgA肾病(IgAN)发病机制的核心。最近的报告表明,过敏性紫癜(HSP)和继发性IgAN存在相似的发病机制。其在其他含IgA的肾小球疾病中的作用仍在研究中。使用最近描述的抗体KM55可在肾小球中检测到它。我们旨在通过对一系列含IgA的肾小球疾病进行免疫染色来评估KM55的作用。
在1例IgAN病例中进行标准化和共定位后,对60例病例进行了研究,包括IgAN、HSP、慢性肝病(CLD)相关的IgAN、其他继发性IgAN、IgA为主/共显性膜增生性肾小球肾炎(MPGN)和狼疮性肾炎,用KM55进行免疫荧光检测。在IgA沉积且组织学表现混淆的病例中,使用KM55来解决诊断难题。
原发性IgAN组(17例)、HSP组(4例)和包括CLD在内的继发性IgAN组(共19例)用KM55检测显示2-3+颗粒状染色,提示为黏膜来源的IgA。相比之下,IgA为主/共显性MPGN组(8例)和狼疮性肾炎组(12例)KM55检测为阴性,提示IgA为全身来源。在IgA沉积且组织学表现混淆的病例中,如膜增生性或弥漫性毛细血管内增生性模式,KM55有助于明确诊断。
讨论/结论:这项横断面研究得出结论,从发病机制角度来看,KM55有助于评估含IgA的肾小球疾病,并且是解决组织病理学特征重叠病例鉴别诊断的实用工具。