There is limited reporting on the longitudinal progression from IMN to systemic lupus erythematosus (SLE) with class V LN, particularly in cases with persistently positive anti-PLA2R antibodies.<h4>Case presentation</h4>A 54-year-old woman presented with nephrotic syndrome in 2016, with initial renal biopsy showing podocyte disease. The gene discussed is PLA2R1; the disease is nephrotic syndrome.