Steroid- and immunosuppressive-resistant nephrotic syndrome in focal and segmental glomerulosclerosis and hyalinosis (FSGSH) with progressive chronic kidney disease: diagnostic and therapeutic challenges

This report presents a complex case of an 18-year-old patient with multiresistant nephrotic syndrome secondary to focal and segmental glomerulosclerosis and hyalinosis (FSGSH), characterized by massive proteinuria (47 to 11 g/24 h), severe hypoproteinemia (albumin 6–17 g/L), immunologic abnormalities (C3, IgM, and C1q deposits), dysregulation of the alternative complement pathway, and a lack of response to standard, combined, and biological therapies. The clinical course included a progressive decline in eGFR, development of anemia, hypercoagulability, infectious complications, and the need for ultrafiltration and hemodialysis. This case reflects the therapeutic limitations in FSGSH and highlights the need for personalized, pathophysiology-based treatment strategies.