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Therapeutic Response of Repository Corticotropin Injection in Treatment of Proteinuria Due to Anca Negative Pauci-immune Crescentic Glomerulonephritis.

Alexander Myint Swan, Htet Htet Kyaw, Zaw Nyein Aye

Corticosteroid and immunosuppressive therapies are considered as first-line therapy for patients with idiopathic Focal Segmental Glomerular Sclerosis (FSGS) associated with clinical features of the nephrotic syndrome. Choosing medications vary individually with underlying causes, renal function and response to the drugs. Here, we report a case of nephrotic syndrome with nephritic component due to pauci-immune crescentic glomerulonephritis treated with the natural Highly Purified Repository Corticotropin Injection (H.P. RCI) as Acthar Gel. A 60-year-old white Hispanic gentleman was diagnosed with biopsy-proved Rapidly Progressive Glomerular Nephritis (RPGN) with serum creatinine 3.6 mg/dl, proteinuria (5800 mg/24 hr), and GFR 18.6 ml/min/1.73 m² and overweight (BMI>29) with underlying diabetes (HbA1C 10.6 %), hypertension (180/90 mmHg) and atrial fibrillation which made him concern to use corticosteroids such as prednisolone and his reduced GFR (<18 ml/min/1.73 m²) favor to avoid calcineurin inhibitors (cyclosporine or tacrolimus) as primary treatment. Therefore, he was given adrenocorticotropin (highly purified ACTH gel 80 U/ml once weekly) injection as a preferred alternative for one and a half year. His proteinuria decreased to 1500 mg/24 hr, and protein/ creatinine ratio decreased from 4795 mg/g to 337 mg/g. Serum creatinine reduced back to 1.9 mg/ dl and GFR 38.6 ml/min/1.73 m². He achieved partial remission which is >50% of the previous baseline with concurrent use of mycophenolate and angiotensin-converting enzyme inhibitors or angiotensin receptor blockers. He has been tolerating well with medications without signs and symptoms of steroid-induced adverse events throughout the treatment with stable proteinuria. These results revealed that the long-term use of repository corticotropin injection gel 80 U/ml once weekly was an effective and safe therapy for patients with idiopathic FSGS and RPGN complicated with diabetic nephropathy.