Abstract / Summary
Abstract Background Lupus podocytopathy (LP) is an entity in systemic lupus erythematosus is characterized by diffuse podocyte foot process effacement, with no immune deposits on renal biopsy. This rare subtype still lacks comprehensive treatment guidelines despite its unique clinical and pathological features; Case presentation We described a 55-year-old female with Han Chinese ethnicity presenting with frequently relapsing nephrotic syndrome and a 10-year medical history. This patient had undergone two previous renal biopsies, indicating lupus nephritis II and focal segmental glomerulosclerosis (FSGS). The current episode was characterized by nephrotic syndrome, acute kidney injury, and positive perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) and anti-MPO antibody. A third renal biopsy confirmed the diagnosis of LP with an FSGS pattern, without pathological features of ANCA-associated vasculitis. Following treatment with corticosteroids, mycophenolate mofetil, and rituximab, her proteinuria and renal function were improved significantly improved. Conclusion We report a rare case of LP with an FSGS pattern and positive MPO-ANCA, presenting with acute kidney injury and a high relapse propensity. In this individual case, ANCA positivity in LP might reflect disease activity, though no obvious correlation with renal pathological lesions was identified. The combination therapy of corticosteroids and other immunosuppressants in this case appears promising for reducing relapse rates in such patients.