Delayed Onset Minimal Change Disease as a Manifestation of Lupus Podocytopathy

Abstract

Lupus podocytopathy (LP) is an uncommon manifestation of systemic lupus erythematosus (SLE) and is not included in the classification of lupus nephritis. The diagnosis of LP is confirmed by the presence of diffuse foot process effacement in the absence of capillary wall deposits with or without mesangial immune deposits in a patient with SLE. Here we describe a 13-year-old female who presented with nephrotic syndrome (NS) seven years after the diagnosis of SLE. The renal function had been stable for seven years since the SLE diagnosis, as manifested by the normal serum creatinine, serum albumin and absence of proteinuria. Renal biopsy showed evidence of minimal change disease without immune complex deposits or features of membranous nephropathy. Renal function was normal. The patient had an excellent response to steroid therapy with remission within two weeks. The patient remained in remission five months later during the most recent follow-up. This report highlights the importance of renal histology to determine the accurate etiology of NS in patients with SLE. Circulating factors, including cytokines such as interleukin 13, may play a role in the pathophysiology of LP and needs to be studied further in future larger studies.

Minimal Change Nephrotic Syndrome in a Child with Systemic Lupus Erythematosus

“…Aly et al . [ 7 ] presented an interesting case of a girl who presented seven years after the diagnosis of SLE with minimal change nephrotic syndrome. She had received rituximab for the initial diagnosis and had been in remission.…”

Tacrolimus Induction Therapy for Nephrotic Syndrome Caused by Minimal Mesangial Lupus Nephritis with Lupus Podocytopathy: A Case-Based Review

“…Of these, only 0.8% are estimated to present with nephrotic syndrome [ 1 ]. Nephrotic syndrome in patients with lupus nephritis has been attributed to immune complex deposition and endocapillary proliferation disrupting the glomerular filtration barrier [ 2 ]. However, nephrotic syndrome has also been observed in minimal mesangial lupus nephritis with electron microscopy findings of diffuse epithelial foot-process effacement.…”