ACTH-induced improvement in the nephrotic syndrome in patients with a variety of diagnoses

Berg, Anna‐Lena; Arnadottir, Margret

doi:10.1093/ndt/gfh110

Cited by 77 publications

(96 citation statements)

References 9 publications

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“…[55][56][57] In case reports from Europe, adrenocorticotropic hormone (ACTH) shows promising results in patients with nephrotic syndrome of various etiologies, including membranous nephropathy, membranoproliferative glomerulonephritis, minimal change disease, and focal segmental glomerulosclerosis. 58,59 In a randomized trial in idiopathic membranous nephropathy conducted by Ponticelli et al, 60 ACTH and cyclophosphamide achieved equal rates of disease remission. Acthar gel, an ACTH formulation available in the United States with Food and Drug Adminstration approval for treating resistant nephrotic syndrome and SLE, may emerge as another potential treatment option for lupus nephritis, particularly class V lupus nephritis.…”

Section: Newer Agents For Lupus Nephritis Will Be Tested In Combinatimentioning

confidence: 99%

Updates on the Treatment of Lupus Nephritis

Bomback

Appel

2010

Journal of the American Society of Nephrology

158

139

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Section: Newer Agents For Lupus Nephritis Will Be Tested In Combinatimentioning

confidence: 99%

Updates on the Treatment of Lupus Nephritis

Bomback

Appel

2010

Journal of the American Society of Nephrology

158

139

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“…In a study by Berg and colleagues (74,75), synthetic adrenocorticotropic hormone (ACTH) administered for 1 yr decreased proteinuria in patients with MN. More recently, Ponticelli et al (76) conducted a randomized pilot study comparing MTP plus a cytotoxic agent versus synthetic ACTH in 32 patients with MN.…”

Section: New Therapiesmentioning

confidence: 99%

Idiopathic Membranous Nephropathy

Fervenza

Sethi

Specks

2008

Clinical Journal of the American Society of Nephrology

133

123

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Idiopathic membranous nephropathy is still the most common glomerular disease associated with nephrotic syndrome. The greater the proteinuria, the greater the long-term risk for renal failure. In addition, patients who have membranous nephropathy with nephrotic syndrome have significant morbidity and mortality, in particular related to thromboembolic and cardiovascular complications. There is no specific treatment for membranous nephropathy. Supportive care with the use of diuretics and angiotensin-converting enzyme inhibitors in combination with angiotensin II receptor blocker is recommended, but these agents have only a limited effect. Immunosuppressive treatment options include the use of corticosteroids, alkylating agents, cyclosporin A, tacrolimus, and mycophenolate mofetil, but their use is controversial, not all have been shown to be effective, and their use can be associated with significant adverse effects. This has resulted in relatively small improvement in the prognosis of membranous nephropathy in the past 30 yr, with up to 40% of patients eventually reaching end-stage renal failure. Agents that offer more complete response rates with lower adverse effects are truly needed. Recent data suggest that B cells play a key role in the pathogenesis of a number of autoimmune diseases including membranous nephropathy and that selective depletion of B cells in humans may be beneficial in preventing the production of pathogenic immunoglobulins and subsequent renal injury.Clin Case PresentationA 32-yr-old man underwent a general medical examination at his primary care physician's office in February 2004. At that time, he was noted to have elevated total cholesterol of 353 mg/dl and triglycerides of 417 mg/dl and was started on simvastatin 40 mg/d. During the course of the next 2 mo, he started developing swelling in his feet and ankles that proceeded to involve his calves. He also developed tenderness on his left calf. A diagnosis of a deep venous thrombosis was made, and he was started on oral anticoagulant therapy. Given the increased lower extremity edema, a urinalysis was conducted, and the patient was found to have significant proteinuria, with a 24-h urine collection showing proteinuria of 9.3 g. The patient had no history of diabetes, macroscopic hematuria, or hypertension. The patient underwent extensive serologic workup including monoclonal protein studies, serum complement levels, anti-neutrophil cytoplasmic antibodies, hepatitis B and C and HIV serology, and anti-nuclear antibody, all of which were negative or in the normal range, and in July 2004, he was referred to our institution for further evaluation. At presentation at the Mayo Clinic, the physical examination was of a healthy-appearing young man. His BP was 136/87 mmHg, pulse rate was 69, weight was 89.8 kg, and body mass index was 29 kg/m 2 . Eyes, nose, and throat examination was normal.There was no lymphadenopathy. Heart examination showed regular rate and rhythm with no murmurs. Lungs were clear to auscultation. Abdomen was soft, with no ...

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“…A synthetic ACTH analogue (tetracosactide, Synacthen R, Novartis Pharmaceuticals, Basel, Switzerland) and a highly purified ACTH gel (H.P. Acthar Gel, Questcor Pharmaceuticals, Inc., Union City, CA) have been used for patients with nephrotic syndrome (26)(27)(28)(29)(30)(31)(32), predominantly in those with membranous nephropathy. To date, the literature describes only five patients (one patient in Europe and four patients in the United States) with nephrotic syndrome due to idiopathic FSGS who have been treated with ACTH; one patient achieved complete response and two patients achieved partial response (27,30,32).…”

Section: Introductionmentioning

confidence: 99%

Treatment of Idiopathic FSGS with Adrenocorticotropic Hormone Gel

Hogan

Bomback

Mehta

et al. 2013

Clinical Journal of the American Society of Nephrology

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SummaryBackground and objectives Adrenocorticotropic hormone (ACTH) has shown efficacy as primary and secondary therapy for nephrotic syndrome due to membranous nephropathy. The data on using ACTH to treat idiopathic FSGS are limited. This report describes our experience using ACTH for nephrotic syndrome due to idiopathic FSGS in the United States.Design, setting, participants, & measurements Twenty-four patients with nephrotic syndrome from idiopathic FSGS were treated with ACTH gel at two academic medical centers between 2009 and 2012, either as part of investigator-initiated pilot studies (n=16) or by prescription for treatment-resistant FSGS (n=8). The primary outcome was remission of proteinuria. The median dose of ACTH was 80 units injected subcutaneously twice weekly. Treatment durations were not uniform.Results Twenty-two patients had received immunosuppression (mean, 2.2 medications) before ACTH therapy. Six patients had steroid-dependent and 15 had steroid-resistant FSGS. At the time of ACTH initiation, the median serum creatinine (interquartile range) was 2.0 (1.1-2.7) mg/dl, estimated GFR was 36 (28-78) ml/min per 1.73 m 2 , and urine protein-to-creatinine ratio was 4595 (2200-8020) mg/g. At the end of ACTH therapy, 7 of 24 patients (29%) experienced remission (n=2 complete remissions, n=5 partial remissions). All remitters had steroid-resistant (n=5) or steroid-dependent (n=2) FSGS. Two responders relapsed during the follow-up period (mean 6 SD, 70631 weeks). Adverse events occurred in 21 of 24 patients, including one episode of new-onset diabetes that resolved after stopping ACTH and two episodes of AKI.Conclusions Response to ACTH treatment among steroid-resistant or steroid-dependent patients with FSGS is low, but ACTH gel may be a viable treatment option for some patients with resistant nephrotic syndrome due to idiopathic FSGS. Further research is necessary to determine which patients will respond to therapy.

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ACTH-induced improvement in the nephrotic syndrome in patients with a variety of diagnoses

Cited by 77 publications

References 9 publications

Updates on the Treatment of Lupus Nephritis

Updates on the Treatment of Lupus Nephritis

Idiopathic Membranous Nephropathy

Treatment of Idiopathic FSGS with Adrenocorticotropic Hormone Gel

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