Skip to main content

Advertisement

SpringerLink
Log in

Glucocorticoids in Myositis: Initiation, Tapering, and Discontinuation

  • Inflammatory Muscle Disease (L Diederichsen and H Chinoy, Section Editors)
  • Published:
Current Rheumatology Reports Aims and scope Submit manuscript

Abstract

Purpose of the review

We discuss the evidence behind the use of glucocorticoids in myositis including a discussion of mechanism of action, dosing, tapering, and duration of therapy as well as glucocorticoid-related adverse events that are particularly important in myositis patients.

Recent findings

Studies showing reduction in mortality rates after the use of glucocorticoids, better outcomes in patients treated with glucocorticoids compared to those who did not, and reduction of inflammation in muscle biopsies provide low level evidence to support use of glucocorticoids in myositis. Early initiation of therapy is associated with better functional outcomes. Use of intravenous methylprednisolone in patients with severe disease may lead to quicker recovery and reduction in long-term glucocorticoid exposure. Steroid-related myopathy and osteoporosis are glucocorticoid side effects that are particularly relevant in myositis.

Summary

The optimal dose and duration of glucocorticoid therapy in myositis currently remain elusive, and this review emphasizes the need for better quality studies in this area.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

Papers of particular interest, published recently, have been highlighted as: • Of importance

  1. Giancane G, Lavarello C, Pistorio A, Oliveira SK, Zulian F, Cuttica R, et al. The PRINTO evidence-based proposal for glucocorticoids tapering/discontinuation in new onset juvenile dermatomyositis patients. Pediatr Rheumatol Online J. 2019;17(1):24. https://doi.org/10.1186/s12969-019-0326-5.

    Article  PubMed  PubMed Central  Google Scholar 

  2. Griggs RC. The current status of treatment for inclusion-body myositis. Neurology. 2006;66(2 Suppl 1):S30–2. https://doi.org/10.1212/01.wnl.0000192262.29924.9e.

    Article  PubMed  Google Scholar 

  3. Boland EW. The effects of cortisone and adrenocorticotropic hormone (ACTH) on certain rheumatic diseases. Calif Med. 1950;72(6):405–14.

    CAS  PubMed  PubMed Central  Google Scholar 

  4. Brunsting LA, Christianson HB, Perry HO. Dermatomyositis; unusual features, complications, and treatment. AMA Arch Derm. 1956;74(6):581–9.

    Article  CAS  Google Scholar 

  5. Vignos PJ Jr, Bowling GF, Watkins MP. Polymyositis; Effect of corticosteroids on final result. Arch Intern Med. 1964;114:263–77. https://doi.org/10.1001/archinte.1964.03860080113012.

    Article  PubMed  Google Scholar 

  6. Winkelmann RK, Mulder DW, Lambert EH, Howard FM Jr, Diessner GR. Course of dermatomyositis-polymyositis: comparison of untreated and cortisone-treated patients. Mayo Clin Proc. 1968;43(8):545–56.

    CAS  PubMed  Google Scholar 

  7. Rose AL, Walton JN. Polymyositis: a survey of 89 cases with particular reference to treatment and prognosis. Brain. 1966;89(4):747–68. https://doi.org/10.1093/brain/89.4.747.

    Article  CAS  PubMed  Google Scholar 

  8. Sheard C Jr. Dermatomyositis. AMA Arch Intern Med. 1951;88(5):640–58. https://doi.org/10.1001/archinte.1951.03810110092009.

    Article  PubMed  Google Scholar 

  9. Eaton LM. The perspective of neurology in regard to polymyositis; a study of 41 cases. Neurology. 1954;4(4):245–63. https://doi.org/10.1212/wnl.4.4.245.

    Article  CAS  PubMed  Google Scholar 

  10. Matsubara S, Hirai S, Sawa Y. Pulsed intravenous methylprednisolone therapy for inflammatory myopathies: evaluation of the effect by comparing two consecutive biopsies from the same muscle. J Neuroimmunol. 1997;76(1–2):75–80. https://doi.org/10.1016/s0165-5728(97)00033-7.

    Article  CAS  PubMed  Google Scholar 

  11. Hardy RS, Raza K, Cooper MS. Therapeutic glucocorticoids: mechanisms of actions in rheumatic diseases. Nat Rev Rheumatol. 2020;16(3):133–44. https://doi.org/10.1038/s41584-020-0371-y.

    Article  PubMed  Google Scholar 

  12. Buttgereit F, Wehling M, Burmester GR. A new hypothesis of modular glucocorticoid actions: steroid treatment of rheumatic diseases revisited. Arthritis Rheum. 1998;41(5):761–7. https://doi.org/10.1002/1529-0131(199805)41:5%3c761::AID-ART2%3e3.0.CO;2-M.

    Article  CAS  PubMed  Google Scholar 

  13. Newton R. Molecular mechanisms of glucocorticoid action: what is important? Thorax. 2000;55(7):603–13. https://doi.org/10.1136/thorax.55.7.603.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Buttgereit F, da Silva JA, Boers M, Burmester GR, Cutolo M, Jacobs J, et al. Standardised nomenclature for glucocorticoid dosages and glucocorticoid treatment regimens: current questions and tentative answers in rheumatology. Ann Rheum Dis. 2002;61(8):718–22. https://doi.org/10.1136/ard.61.8.718.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Mozaffar T, Pestronk A. Myopathy with anti-Jo-1 antibodies: pathology in perimysium and neighbouring muscle fibres. J Neurol Neurosurg Psychiatry. 2000;68(4):472–8. https://doi.org/10.1136/jnnp.68.4.472.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Nagaraju K, Raben N, Loeffler L, Parker T, Rochon PJ, Lee E, et al. Conditional up-regulation of MHC class I in skeletal muscle leads to self-sustaining autoimmune myositis and myositis-specific autoantibodies. Proc Natl Acad Sci U S A. 2000;97(16):9209–14. https://doi.org/10.1073/pnas.97.16.9209.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  17. Ulfgren AK, Grundtman C, Borg K, Alexanderson H, Andersson U, Harris HE, et al. Down-regulation of the aberrant expression of the inflammation mediator high mobility group box chromosomal protein 1 in muscle tissue of patients with polymyositis and dermatomyositis treated with corticosteroids. Arthritis Rheum. 2004;50(5):1586–94. https://doi.org/10.1002/art.20220.

    Article  CAS  PubMed  Google Scholar 

  18. • Hoff LS, de Souza FHC, Miossi R, Shinjo SK. Long-term effects of early pulse methylprednisolone and intravenous immunoglobulin in patients with dermatomyositis and polymyositis. Rheumatology (Oxford). 2021. https://doi.org/10.1093/rheumatology/keab597. (Recent retrospective study showing higher rates of complete clinical response in patients treated with pulse dose steroids.)

    Article  Google Scholar 

  19. Matsubara S, Sawa Y, Takamori M, Yokoyama H, Kida H. Pulsed intravenous methylprednisolone combined with oral steroids as the initial treatment of inflammatory myopathies. J Neurol Neurosurg Psychiatry. 1994;57(8):1008. https://doi.org/10.1136/jnnp.57.8.1008.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. Yanagisawa T, Sueishi M, Nawata Y, Akimoto T, Nozaki T, Koike T, et al. Methylprednisolone pulse therapy in dermatomyositis. Dermatologica. 1983;167(1):47–51. https://doi.org/10.1159/000249745.

    Article  CAS  PubMed  Google Scholar 

  21. Bolosiu HD, Man L, Rednic S. The effect of methylprednisolone pulse therapy in polymyositis/dermatomyositis. Adv Exp Med Biol. 1999;455:349–57. https://doi.org/10.1007/978-1-4615-4857-7_54.

    Article  CAS  PubMed  Google Scholar 

  22. Rouster-Stevens KA, Gursahaney A, Ngai KL, Daru JA, Pachman LM. Pharmacokinetic study of oral prednisolone compared with intravenous methylprednisolone in patients with juvenile dermatomyositis. Arthritis Rheum. 2008;59(2):222–6. https://doi.org/10.1002/art.23341.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Henriksson KG, Lindvall B. Polymyositis and dermatomyositis 1990–diagnosis, treatment and prognosis. Prog Neurobiol. 1990;35(3):181–93. https://doi.org/10.1016/0301-0082(90)90026-d.

    Article  CAS  PubMed  Google Scholar 

  24. Joffe MM, Love LA, Leff RL, Fraser DD, Targoff IN, Hicks JE, et al. Drug therapy of the idiopathic inflammatory myopathies: predictors of response to prednisone, azathioprine, and methotrexate and a comparison of their efficacy. Am J Med. 1993;94(4):379–87. https://doi.org/10.1016/0002-9343(93)90148-i.

    Article  CAS  PubMed  Google Scholar 

  25. Espinosa-Ortega F, Holmqvist M, Dastmalchi M, Lundberg IE, Alexanderson H. Factors associated with treatment response in patients with idiopathic inflammatory myopathies. A registry-based study. Arthritis Care Res (Hoboken). 2020. https://doi.org/10.1002/acr.24498.

  26. Henriksson KG, Sandstedt P. Polymyositis--treatment and prognosis A study of 107 patients. Acta Neurol Scand. 1982;65(4):280–300. https://doi.org/10.1111/j.1600-0404.1982.tb03087.x.

    Article  CAS  PubMed  Google Scholar 

  27. Nzeusseu A, Brion F, Lefebvre C, Knoops P, Devogelaer JP, Houssiau FA. Functional outcome of myositis patients: can a low-dose glucocorticoid regimen achieve good functional results? Clin Exp Rheumatol. 1999;17(4):441–6.

    CAS  PubMed  Google Scholar 

  28. Myles AB, Bacon PA, Daly JR. Single daily dose corticosteroid treatment Effect on adrenal function and therapeutic efficacy in various diseases. Ann Rheum Dis. 1971;30(2):149–53.

    Article  CAS  Google Scholar 

  29. Uchino M, Araki S, Yoshida O, Uekawa K, Nagata J. High single-dose alternate-day corticosteroid regimens in treatment of polymyositis. J Neurol. 1985;232(3):175–8. https://doi.org/10.1007/BF00313897.

    Article  CAS  PubMed  Google Scholar 

  30. Uchino M, Yamashita S, Uchino K, Hara A, Koide T, Suga T, et al. Long-term outcome of polymyositis treated with high single-dose alternate-day prednisolone therapy. Eur Neurol. 2012;68(2):117–21. https://doi.org/10.1159/000338474.

    Article  CAS  PubMed  Google Scholar 

  31. Axelrod L. Glucocorticoid therapy. Medicine (Baltimore). 1976;55(1):39–65. https://doi.org/10.1097/00005792-197601000-00003.

    Article  CAS  Google Scholar 

  32. Oddis CV, Medsger TA Jr. Relationship between serum creatine kinase level and corticosteroid therapy in polymyositis-dermatomyositis. J Rheumatol. 1988;15(5):807–11.

    CAS  PubMed  Google Scholar 

  33. • van de Vlekkert J, Hoogendijk JE, de Haan RJ, Algra A, van der Tweel I, van der Pol WL, et al. Oral dexamethasone pulse therapy versus daily prednisolone in sub-acute onset myositis, a randomised clinical trial. Neuromuscul Disord. 2010;20(6):382–9. https://doi.org/10.1016/j.nmd.2010.03.011. (One of the few randomized controlled trials conducted to assess different glucocorticoid regimens in myositis.)

    Article  PubMed  Google Scholar 

  34. Metzger AL, Bohan A, Goldberg LS, Bluestone R, Pearson CM. Polymyositis and dermatomyositis: combined methotrexate and corticosteroid therapy. Ann Intern Med. 1974;81(2):182–9. https://doi.org/10.7326/0003-4819-81-2-182.

    Article  CAS  PubMed  Google Scholar 

  35. Bunch TW. Prednisone and azathioprine for polymyositis: long-term followup. Arthritis Rheum. 1981;24(1):45–8. https://doi.org/10.1002/art.1780240107.

    Article  CAS  PubMed  Google Scholar 

  36. Sokoloff MC, Goldberg LS, Pearson CM. Treatment of corticosteroid-resistant polymyositis with methotrexate. Lancet. 1971;1(7688):14–6. https://doi.org/10.1016/s0140-6736(71)80005-3.

    Article  CAS  PubMed  Google Scholar 

  37. • Loarce-Martos J, Lilleker JB, Alder E, Goode J, Chinoy H. Perspectives on glucocorticoid usage in patients with adult inflammatory myopathy. Clin Rheumatol. 2021. https://doi.org/10.1007/s10067-021-05767-8. (Recent survey study reporting perspectives of the patients with myositis on glucocorticoid use.)

    Article  PubMed  Google Scholar 

  38. Azali P, Barbasso Helmers S, Kockum I, Olsson T, Alfredsson L, Charles PJ, et al. Low serum levels of vitamin D in idiopathic inflammatory myopathies. Ann Rheum Dis. 2013;72(4):512–6. https://doi.org/10.1136/annrheumdis-2012-201849.

    Article  CAS  PubMed  Google Scholar 

  39. Cox M, Sandler RD, Matucci-Cerinic M, Hughes M. Bone health in idiopathic inflammatory myopathies. Autoimmun Rev. 2021;20(4):102782. https://doi.org/10.1016/j.autrev.2021.102782.

    Article  CAS  PubMed  Google Scholar 

  40. Vincze A, Bodoki L, Szabo K, Nagy-Vincze M, Szalmas O, Varga J, et al. The risk of fracture and prevalence of osteoporosis is elevated in patients with idiopathic inflammatory myopathies: cross-sectional study from a single Hungarian center. BMC Musculoskelet Disord. 2020;21(1):426. https://doi.org/10.1186/s12891-020-03448-2.

    Article  PubMed  PubMed Central  Google Scholar 

  41. Gupta A, Gupta Y. Glucocorticoid-induced myopathy: pathophysiology, diagnosis, and treatment. Indian J Endocrinol Metab. 2013;17(5):913–6. https://doi.org/10.4103/2230-8210.117215.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  42. Walsh M, Merkel PA, Peh CA, Szpirt WM, Puechal X, Fujimoto S, et al. Plasma exchange and glucocorticoids in severe ANCA-associated vasculitis. N Engl J Med. 2020;382(7):622–31. https://doi.org/10.1056/NEJMoa1803537-42.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  43. Stone JH, Tuckwell K, Dimonaco S, Klearman M, Aringer M, Blockmans D, et al. Trial of tocilizumab in giant-cell arteritis. N Engl J Med. 2017;377(4):317–28. https://doi.org/10.1056/NEJMoa1613849.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Section of Rheumatology, Department of Medicine, University of Chicago, Chicago, IL, USA

    Didem Saygin

  2. Division of Rheumatology and Clinical Immunology, Department of MedicineSchool of MedicineBiomedical Science Tower, University of Pittsburgh School of Medicine, South 705, Biomedical Science Tower, 3500 Terrace Street, Pittsburgh, PA, 15261, USA

    Chester V. Oddis

Authors
  1. Didem Saygin
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Chester V. Oddis
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Chester V. Oddis.

Ethics declarations

Human and Animal Rights and Informed Consent.

This article does not contain any studies with human or animal subjects performed by any of the authors.

Conflict of Interest

The authors declare no competing interests.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

This article is part of the Topical Collection on Inflammatory Muscle Disease

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Saygin, D., Oddis, C.V. Glucocorticoids in Myositis: Initiation, Tapering, and Discontinuation. Curr Rheumatol Rep 24, 47–53 (2022). https://doi.org/10.1007/s11926-022-01060-y

Download citation

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11926-022-01060-y

Keywords

Search

Navigation