Repository Corticotropin Injection for Treatment of Idiopathic Inflammatory Myopathies

Abstract

Idiopathic inflammatory myopathies are a group of systemic autoimmune diseases that involve inflammation of skeletal muscle. The two most common forms are dermatomyositis and polymyositis, the former of which entails a skin component. There are few approved therapeutics available for treatment of this group of diseases and the first-line therapy is usually corticosteroid treatment. Considering that a large proportion of patients do not respond to or cannot tolerate corticosteroids, additional treatments are required. There are second-line therapies available, but many patients are also refractory to those options. H.P. Acthar® Gel (repository corticotropin injection [RCI]) is a melanocortin peptide that can induce steroid-dependent effects and steroid-independent effects. Herein, we present a series of cases that involved the use of RCI in the management of dermatomyositis and polymyositis. RCI treatments resulted in improvement in three of four patients, despite failure with previous therapies. The use of RCI did not exacerbate any comorbidity and no significant changes in blood pressure, weight, or glycemic control were observed. Overall, these results are encouraging and suggest that randomized, controlled clinical trials applying RCI to dermatomyositis and polymyositis are warranted.

Efficacy and safety of adrenocorticotropic hormone gel in refractory dermatomyositis and polymyositis

“…A previous retrospective case series using the same dosing regimen employed in our trial noted similar response rates, and a follow-up retrospective study of 24 patients with myositis treated with RCI at different clinical centres showed 58.3% response rates 3 13. Another small retrospective case series demonstrated a steroid dose reduction and similar efficacy in three of four adult patients with refractory DM/PM including one patient with the anti-SRP autoantibody who failed IVIG and rituximab 14. Again no differences were noted in the response rates of DM versus PM or rates of response to muscle weakness versus cutaneous rashes in previous retrospective studies.…”

Industry Payments to Physician Specialists Who Prescribe Repository Corticotropin

“…Among these conditions, controlled clinical trial data only support corticotropin use to treat exacerbations of multiple sclerosis, 18 , 19 although it is no more effective than methylprednisolone. The use of corticotropin for rheumatoid arthritis, 39 dermatomyositis and polymyositis, 40 , 41 , 42 systemic lupus erythematosus, 25 , 43 and nephrotic syndrome 44 , 45 , 46 , 47 , 48 is largely supported by small uncontrolled studies or case series.…”

Biologics in refractory myositis: experience in juvenile vs. adult myositis; part II: emerging biologic and other therapies on the horizon

“…There is limited and patchy research on the use of ACTH analogs in adult and childhood myositis. Aarat Patel et al reported their experience with 4 steroid-resistant and refractory myositis patients who were treated with RCI [42]. Malignancy was ruled out in all the four patients.…”

“…Aarat Patel et al reported their experience with 4 steroid-resistant and refractory myositis patients who were treated with RCI [42]. Malignancy was ruled out in all the four patients.…”