Abstract

BackgroundLupus 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.MethodologyA 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).ResultsEighty-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.ConclusionsLP is a spectrum of p-SLE, not an association as it is related to disease activity and its initial presentation.

Highlights

  • Systemic lupus erythematosus (SLE) is an inflammatory autoimmune disease affecting multi-systems with periods of activities and remissions [1, 2]

  • Five biopsies were normal (MCD) and two showed focal segmental sclerosis (FSGN) that did not meet any known classification of lupus nephritis (LN)

  • Lupus podocytopathy (LP) is a spectrum of p-systemic lupus erythematosus (SLE), not an association as it is related to disease activity and its initial presentation

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Summary

Introduction

Systemic lupus erythematosus (SLE) is an inflammatory autoimmune disease affecting multi-systems with periods of activities and remissions [1, 2]. Pathology Society (ISN/RPS) in 2018, emphasized that immune complex aggregation is central to LN and universally present in all subclasses [5]. The increasingly recognized phenomenon of apparent minimal change disease (MCD) and focal segmental glomerular sclerosis (FSGS) without significant immune deposits in a patient with SLE is termed lupus podocytopathy (LP) [6]. The diagnosis of LP in a patient with SLE and nephrotic syndrome is based on the finding of diffuse foot process effacement in the absence of peripheral capillary wall immune deposits [7]. 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

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