Resistant Nephrotic Syndrome: Review of Trials Using ACTH and a Case Series of Six Patients Treated with ACTHar
Jaime Wolf
Department of Internal Medicine, Lankenau Medical Center, United States.
Vishnu Patel
Department of Internal Medicine, Lankenau Medical Center, United States.
Rishi Prasad
Radnor High School, United States.
Geoffrey Teehan *
Division of Nephrology, Department of Internal Medicine, Lankenau Medical Center, United States.
*Author to whom correspondence should be addressed.
Abstract
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.
Keywords: Nephrotic syndrome, proteinuria, ACTHar