Abstract
BACKGROUND: Glomerulopathy involves damage to the glomerular filtration barrier for several reasons, resulting in idiopathic nephrotic syndrome (NS). Treatment options are limited and often include steroids with varying levels of response. CASE PRESENTATION: A 7-year-old male with a history of NS at age 2 years that developed following a respiratory tract infection was found to have a heterozygous variant of uncertain significance in COL4A4 and TRPC6 genes. Biopsy findings included podocytopathy and changes in the basement membrane. Upon initial response to steroids, the patient was treated with a brief course of anakinra followed by adalimumab for > 2 years as steroid-sparing biological response modifiers. After a gradual taper, the patient remains in remission and has not received treatment in the last 12 months. CONCLUSIONS: This case shows the complex nature of biologically predetermined cascading events in the emergence of glomerular disease with environmental triggers and genetic factors. Downregulation of somatic tissue-driven proinflammatory milieu originating from the constituents of the glomerular microenvironment can help in recovery from emerging podocytopathy. Blocking tumor necrosis factor-α early in the disease course, even temporarily, may allow time for the de novo regenerative process to prevail. Additional research is warranted to test this hypothesis and minimize steroid use.