Huerta Ana, Bomback Andrew S, Liakopoulos Vassilis, Palanisamy Amudha, Stokes M Barry, D'Agati Vivette D, Radhakrishnan Jai, Markowitz Glen S, Appel Gerald B
Department of Nephrology, Hospital Universitario 12 de Octubre, Universidad Complutense de Madrid, Madrid, Spain.
Nephrol Dial Transplant. 2012 Jun;27(6):2337-42. doi: 10.1093/ndt/gfr663. Epub 2011 Dec 29.
In the setting of an IgG-dominant immune complex-mediated glomerulonephritis, there are multiple pathological findings that strongly suggest the diagnosis of lupus nephritis (LN) including (i) 'full-house' immunofluorescence staining for IgG, IgM, IgA, C3 and C1; (ii) extraglomerular immune deposits; (iii) combined mesangial, subendothelial and subepithelial immune deposits and (iv) the presence of endothelial tubuloreticular inclusions. We report four female adult patients with renal biopsy findings which are highly suggestive of LN but without extrarenal signs, symptoms or serologies of systemic lupus erythematosus at the time of biopsy or over a mean follow-up period of 3 years. Despite aggressive therapy, outcomes were poor in this small cohort. We refer to these cases as renal-limited 'lupus-like' nephritis.
在以IgG为主的免疫复合物介导的肾小球肾炎背景下,有多项病理表现强烈提示狼疮性肾炎(LN)的诊断,包括:(i)IgG、IgM、IgA、C3和C1的“满堂亮”免疫荧光染色;(ii)肾小球外免疫沉积物;(iii)系膜、内皮下和上皮下免疫沉积物并存;以及(iv)内皮细胞管形网状包涵体的存在。我们报告了4例成年女性患者,其肾活检结果高度提示LN,但在活检时或平均3年的随访期内无系统性红斑狼疮的肾外体征、症状或血清学表现。尽管进行了积极治疗,但这个小队列的预后较差。我们将这些病例称为肾脏局限性“狼疮样”肾炎。