Nephrology Unit, Pediatric Department, Tanta University, El-Geesh Street, Tanta, Egypt.
Pediatr Rheumatol Online J. 2021 Apr 26;19(1):57. doi: 10.1186/s12969-021-00547-9.
Lupus podocytopathy (LP) is a renal affection described in systemic lupus erythematosus (SLE) patients with nephrotic range proteinuria, characterized by diffuse foot process effacement without immune deposits and glomerular proliferation. This study describes LP, its pathological features and outcomes of pediatric (p-SLE) patients in comparison to the usual lupus nephritis (LN) cases.
A retrospective cohort study conducted on a 10-year registration (2010-2019) of 140 p-SLE patients at the Pediatric Department, Tanta University. Histopathological analysis with light microscopy (LM) and immunofluorescence (IF) of all renal biopsies were evaluated according to the International Society of Nephrology Renal Pathology Society (ISN/RPS) grading system. In addition, some biopsies were examined with electron microscopy (EM).
Eighty-six p-SLE cases (61.4%) had renal involvement; seventy-nine biopsies (91.86%) of them met the classification criteria of LN as defined by ISN/RPS system. Five biopsies were normal (MCD) and two showed focal segmental sclerosis (FSGN) that did not meet any known classification of LN. Hence, they were reevaluated using EM that revealed diffuse effaced podocytes without glomerular sub-epithelial, endocapillary or basement membrane immune deposits, and were classified as having lupus podocytopathy, representing (8.14%) of all LN biopsies. Those seven cases showed good response to steroids with a complete remission duration of 3.40 ± 1.95 weeks. However, some case had 1-3 relapses during the duration of follow up.
LP is a spectrum of p-SLE, not an association as it is related to disease activity and its initial presentation.
狼疮性足细胞病（LP）是一种在表现为肾病范围蛋白尿的系统性红斑狼疮（SLE）患者中描述的肾脏病变，其特征为弥漫性足突融合而无免疫沉积物和肾小球增殖。本研究描述了 LP 的病理特征及其在儿科（p-SLE）患者中的表现，并与常见的狼疮性肾炎（LN）病例进行了比较。
对坦塔大学儿科系 10 年（2010-2019 年）登记的 140 例 p-SLE 患者进行回顾性队列研究。根据国际肾脏病学会肾脏病理学会（ISN/RPS）分级系统，对所有肾活检进行光镜（LM）和免疫荧光（IF）的组织病理学分析。此外，一些活检还进行了电子显微镜（EM）检查。
86 例 p-SLE 患者（61.4%）有肾脏受累；其中 79 例（91.86%）活检符合 ISN/RPS 系统定义的 LN 分类标准。5 例活检正常（MCD），2 例表现为局灶节段性肾小球硬化（FSGN），不符合任何已知的 LN 分类。因此，使用 EM 重新评估，结果显示弥漫性足突融合，无肾小球下皮、毛细血管内或基底膜免疫沉积物，被归类为狼疮性足细胞病，占所有 LN 活检的（8.14%）。这 7 例患者对类固醇反应良好，完全缓解持续时间为 3.40±1.95 周。然而，在随访期间，一些患者有 1-3 次复发。
LP 是 p-SLE 的一个谱系，不是一种关联，因为它与疾病活动和初始表现有关。
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