Resistant Nephrotic Syndrome: Review of Trials Using ACTH and a Case Series of Six Patients Treated with ACTHar

Nephrotic syndrome (NS) is associated with edema, proteinuria, hyperlipidemia, and an increased risk for deep vein thrombosis. Therapy may include glucocorticoids, immunosuppressive therapy, and/or chemotherapy. Each is associated with significant toxicity. Adrenocorticotrophic hormone (ACTH) has been approved for treating NS since the 1950s. Both the synthetic form of ACTH (cosyntropin-ACTH 1-24) and ACTHar (ACTH1-39), a synthetic gel isolated from porcine pituitary extracts, have been used successfully in treating NS but fewer than 100 published cases of ACTHar therapy are available in the literature. Membranous nephropathy is the best studied entity. Our case series adds six new cases to the literature and strengthens the growing consensus that ACTHar may be effective in treating focal segmental glomerulosclerosis.