Treating refractory dermatomyositis or polymyositis with adrenocorticotropic hormone gel: a retrospective case series

Todd Levine, Todd Levine

Abstract

Background: Effective and tolerable treatment options for patients with dermatomyositis and polymyositis are limited. This retrospective case review describes treatment with adrenocorticotropic hormone (ACTH) gel in five patients who experienced a disease exacerbation and either failed or were unable to tolerate the side effects of previous therapy with steroids, intravenous immunoglobulins, and steroid-sparing drugs.

Methods: Patients received ACTH gel subcutaneous injections of 80 U (1 mL) twice weekly (four patients) or once weekly (one patient) over the course of 12 weeks for short-term treatment of symptom exacerbations. Manual muscle testing using the Medical Research Council scale was assessed at baseline and at 3 months.

Results: Improvement was seen in all patients, including improved muscle strength, decreased pain, and resolution of skin involvement. All patients tolerated the treatment well, and no significant side effects occurred.

Conclusion: The treatment of dermatomyositis and polymyositis is an approved use for ACTH gel, and these anecdotal reports would suggest consideration of ACTH gel as a therapeutic option. Further investigation is warranted.

Keywords: adrenocorticotropic hormone gel; dermatomyositis; intravenous immunoglobulins; polymyositis; steroids.

References

    1. Dalakas MC. Immunotherapy of myositis: issues, concerns and future prospects. Nat Rev Rheumatol. 2010;6(3):129–137.
    1. Marie I, Mouthon L. Therapy of polymyositis and dermatomyositis. Autoimmun Rev. 2011;11(1):6–13.
    1. Patwa HS, Chaudhry V, Katzberg H, Rae-Grant AD, So YT. Evidence-based guideline: intravenous immunoglobulin in the treatment of neuromuscular disorders: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2012;78(13):1009–1015.
    1. Lin RY, Rodriguez-Baez G, Bhargave GA, Lin H. Intravenous gammaglobulin- associated renal impairment reported to the FDA: 2004–2009. Clin Nephrol. 2011;76(5):365–372.
    1. Levine TD. Rituximab in the treatment of dermatomyositis: an open-label pilot study. Arthritis Rheum. 2005;52(2):601–607.
    1. Oddis CV, Reed AM, Aggarwal R, et al. Rituximab in the treatment of refractory adult and juvenile dermatomyositis (DM) and adult polymyositis (PM) – the RIM Study [L13]. Presented at the American College of Rheumatology/Association of Rheumatology Health Professionals annual scientific meeting; November 6–10, 2010; Atlanta, GA.
    1. Questcor Pharmaceuticals, Inc. HP Acthar® Gel (repository corticotropin injection, full prescribing information) Hayward, CA: Questcor Pharmaceuticals, Inc; 2011.
    1. Catania A, Lonati C, Sordi A, Carlin A, Leonardi P, Gatti S. The melanocortin system in control of inflammation. Scientific World Journal. 2010;10:1840–1853.
    1. Baram TZ, Mitchell WG, Tournay A, Snead OC, Hanson RA, Horton EJ. High-dose corticotropin (ACTH) versus prednisone for infantile spasms: a prospective, randomized, blinded study. Pediatrics. 1996;97(3):375–379.
    1. Bomback AS, Tumlin JA, Baranski J, et al. Treatment of nephrotic syndrome with adrenocorticotropic hormone (ACTH) gel. Drug Des Devel Ther. 2011;5:147–153.
    1. Simsarian JP, Saunders C, Smith DM. Five-day regimen of intramuscular or subcutaneous self-administered adrenocorticotropic hormone gel for acute exacerbations of multiple sclerosis: a prospective, randomized, open-label pilot trial. Drug Des Devel Ther. 2011;5:381–389.
    1. Rider LG. Outcome assessment in the adult and juvenile idiopathic inflammatory myopathies. Rheum Dis Clin North Am. 2002;28(4):935–977.
    1. Oddis CV, Rider LG, Reed AM, et al. International consensus guidelines for trials of therapies in the idiopathic inflammatory myopathies. Arthritis Rheum. 2005;52(9):2607–2615.
    1. Rider LG, Giannini EH, Brunner HI, et al. International consensus on preliminary definitions of improvement in adult and juvenile myositis. Arthritis Rheum. 2004;50(7):2281–2290.
    1. Lohmann Siegel K, Hicks JE, Koziol DE, Gerber LH, Rider LG. Walking ability and its relationship to lower-extremity muscle strength in children with idiopathic inflammatory myopathies. Arch Phys Med Rehabil. 2004;85(5):767–771.
    1. Uchikawa K, Liu M, Hanayama K, Tsuji T, Fujiwara T, Chino N. Functional status and muscle strength in people with Duchenne muscular dystrophy living in the community. J Rehabil Med. 2004;36(3):124–129.
    1. Rider LG, Koziol D, Giannini EH, et al. Validation of manual muscle testing and a subset of eight muscles for adult and juvenile idiopathic inflammatory myopathies. Arthritis Care Res (Hoboken) 2010;62(4):465–472.

Source: PubMed

3
Tilaa