Sathi Satyanand, Sharma Alok, Garg Anil Kumar, Saini Virendra Singh, Singh Manoj Kumar, Vohra Devinder, Trivedi Arvind
Department of Medicine, S.M.M.H. Government Medical College, Saharanpur, Uttar Pradesh, India.
Department of Renal Pathology and Electron Microscopy, Dr Lal Path-lab Limited, New Delhi, India.
Case Rep Nephrol. 2021 Dec 28;2021:9043003. doi: 10.1155/2021/9043003. eCollection 2021.
Full-house immunofluorescence and endothelial tubuloreticular inclusions are known as characteristic features of lupus nephritis. However, both features are not pathognomonic for lupus nephritis. A kidney biopsy specimen showing full-house immunofluorescence pattern in the absence of autoantibodies and classical clinical features of Systemic Lupus Erythematosus (SLE) is now considered as nonlupus full-house nephropathy (FHN). Nonlupus FHN may be idiopathic or due to other disease processes known as secondary nonlupus FHN. Here, we report the case of a 36-year-old female who presented with nephrotic proteinuria with bland urine sediment. Additional analyses revealed normal serum antinuclear antibody (ANA), normal anti-double-stranded DNA (anti-dsDNA) antibodies, and normal serum C3 and C4 levels. A renal biopsy showed a normal-appearing glomerulus without any proliferation or capillary wall thickening and widespread glomerular immune deposits (full-house effect; IgA, IgG, IgM, C3, and C1Q) on direct immunofluorescence. Renal electron microscopy showed diffuse effacement of visceral epithelial cell foot processes and mesangial electron dense deposits. The patient was diagnosed as nonlupus FHN. There is a controversial role of steroids and other immunosuppressive drugs in the treatment of nonlupus FHN patients, but our case patient responded favourably to steroid therapy. The term nonlupus FHN can be used as an umbrella term for patients who do not satisfy the clinical and serological criteria of SLE.
满堂亮免疫荧光和内皮管型网状包涵体是狼疮性肾炎的特征性表现。然而，这两种表现并非狼疮性肾炎所特有。在没有自身抗体和系统性红斑狼疮（SLE）典型临床特征的情况下，肾脏活检标本显示满堂亮免疫荧光模式，现在被认为是非狼疮性满堂亮肾病（FHN）。非狼疮性FHN可能是特发性的，也可能是由其他疾病过程引起的，称为继发性非狼疮性FHN。在此，我们报告一例36岁女性患者，表现为肾病性蛋白尿，尿沉渣正常。进一步分析显示血清抗核抗体（ANA）正常、抗双链DNA（抗dsDNA）抗体正常、血清C3和C4水平正常。肾脏活检显示肾小球外观正常，无任何增殖或毛细血管壁增厚，直接免疫荧光显示广泛的肾小球免疫沉积物（满堂亮效应；IgA、IgG、IgM、C3和C1Q）。肾脏电子显微镜检查显示脏层上皮细胞足突弥漫性消失和系膜电子致密沉积物。该患者被诊断为非狼疮性FHN。类固醇和其他免疫抑制药物在非狼疮性FHN患者治疗中的作用存在争议，但我们的病例患者对类固醇治疗反应良好。非狼疮性FHN这一术语可作为不符合SLE临床和血清学标准患者的统称。
