Is podocytopathy another image of renal affection in p-SLE?

Abstract

Background 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. Methodology 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). Results 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. Conclusions LP is a spectrum of p-SLE, not an association as it is related to disease activity and its initial presentation.

Lupus podocytopathy and antiphospholipid syndrome in a child with SLE: A case report and literature review

“…LP accounts for 1.33% of all LN biopsies in adults (9), representing 8.14% in children (21). Our data showed LP in <1% of all children with LN biopsies (unpublished).…”

“…To date, eleven pediatric cases of LP have been reported in PubMed. Four were case reports including our patient (Table 2), and a cohort study included seven patients (21)(22)(23)(24), which suggests that it is a very rare complication of SLE in children. All children with LP have been girls and present with NS (21)(22)(23)(24).…”

“…Four were case reports including our patient (Table 2), and a cohort study included seven patients (21)(22)(23)(24), which suggests that it is a very rare complication of SLE in children. All children with LP have been girls and present with NS (21)(22)(23)(24). Kidney biopsy findings revealed MCD in eight patients and FSGS in three patients (21)(22)(23)(24).…”

Delayed Onset Minimal Change Disease as a Manifestation of Lupus Podocytopathy

“…In the study by Hu et al [21] who examined adult cases of SLE, 50 out of 3750 (1.33%) renal biopsies met the definition of lupus podocytopathy. In the retrospective pediatric study by Abdelnabi et al, the incidence of lupus podocytopathy was found to be as high as 8.1% [23].…”

“…However, in rare instances, SLE patients with NS can have renal biopsy findings of normal appearing glomeruli, with or without mesangial proliferation, on light microscopy; the absence of subepithelial or subendothelial deposits on immunofluorescence and electron microscopy; and diffuse foot process effacement on electron microscopy. This pattern, termed LP, represents about 1% of LN biopsies [21,22]; however, one report found that about 8% of patients with pediatric LN may have LP [23].…”