Updates in Treatment of Refractory Inflammatory Myositis

Authors

  • Kun Huang, MD, PhD, FRCPC Department of Medicine, University of British Columbia, Vancouver, British Columbia Division of Rheumatology, Vancouver General Hospital, Vancouver, British Columbia Division of Rheumatology, Surrey Memorial Hospital, Surrey, British Columbia Arthritis Research Canada, Vancouver, British Columbia
  • Aidan Pye, MD Department of Medicine, University of British Columbia, Vancouver, British Columbia

DOI:

https://doi.org/10.58931/crt.2024.1145

Abstract

Idiopathic immune myopathies (IIM), also known as myositis, are a heterogeneous group of autoimmune diseases with varying phenotypes, prognoses, and treatment responses. They are primarily characterized by muscle inflammation, however, many patients have extramuscular involvement including skin rash, arthritis, interstitial lung disease (ILD), cardiomyopathy, and gastrointestinal dysmotility. The discovery of myositis-specific autoantibodies (MSAs) has been a major advancement in the field of IIMs, shaping the new landscape of the clinical, phenotypical, histological, and serological correlations. Based on this discovery, IIM can be more specifically classified into dermatomyositis (DM) (including amyopathic DM), antisynthetase syndrome (ASyS), immune-mediated necrotizing myopathy (IMNM), inclusion body myositis (IBM), polymyositis (PM), and overlap myositis (OM). An increasing number of histological studies have revealed a misdiagnosis of PM because many patients who were previously diagnosed with PM were later reclassified to other forms of IIM, including IBM, IMNM, ASyS, or DM without a rash.

Author Biographies

Kun Huang, MD, PhD, FRCPC, Department of Medicine, University of British Columbia, Vancouver, British Columbia Division of Rheumatology, Vancouver General Hospital, Vancouver, British Columbia Division of Rheumatology, Surrey Memorial Hospital, Surrey, British Columbia Arthritis Research Canada, Vancouver, British Columbia

Dr. Kun Huang is a rheumatologist at Surrey Memorial Hospital and a myositis specialist at the Mary Pack Arthritis Program at Vancouver Coastal Health. She is a clinical assistant professor at the University of British Columbia and a clinical investigator at the Arthritis Research Canada. She has an academic focus in inflammatory myositis therapies and outcome.

Aidan Pye, MD, Department of Medicine, University of British Columbia, Vancouver, British Columbia

Dr. Aidan Pye is a first-year internal medicine resident at the University of British Columbia with an interest in rheumatology. He completed his undergraduate degree in chemistry at the University of Victoria and MD at the University of British Columbia.

References

Lundberg IE, Fujimoto M, Vencovsky J, Aggarwal R, Holmqvist M, Christopher-Stine L, et al. Idiopathic inflammatory myopathies. Nat Rev Dis Primers. 2021;7(1):86. DOI: https://doi.org/10.1038/s41572-021-00321-x

Loarce-Martos J, Lilleker JB, Parker M, McHugh N, Chinoy H. Polymyositis: is there anything left? A retrospective diagnostic review from a tertiary myositis centre. Rheumatology (Oxford). 2021;60(7):3398-403. DOI: https://doi.org/10.1093/rheumatology/keaa801

Oddis CV, Reed AM, Aggarwal R, Rider LG, Ascherman DP, Levesque MC, et al. Rituximab in the treatment of refractory adult and juvenile dermatomyositis and adult polymyositis: a randomized, placebo-phase trial. Arthritis Rheum. 2013;65(2):314-24. DOI: https://doi.org/10.1002/art.37754

Aggarwal R, Charles-Schoeman C, Schessl J. Trial of Intravenous Immune Globulin in Dermatomyositis. Reply. N Engl J Med. 2023;388(1):94-5. DOI: https://doi.org/10.1056/NEJMc2214285

Oldroyd AGS, Callen JP, Chinoy H, Chung L, Fiorentino D, Gordon P, et al. International Guideline for Idiopathic Inflammatory Myopathy-Associated Cancer Screening: an International Myositis Assessment and Clinical Studies Group (IMACS) initiative. Nat Rev Rheumatol. 2023;19(12):805-17. DOI: https://doi.org/10.1038/s41584-023-01045-w

Watanabe E, Gono T, Kuwana M, Terai C. Predictive factors for sustained remission with stratification by myositis-specific autoantibodies in adult polymyositis/dermatomyositis. Rheumatology (Oxford). 2020;59(3):586-93. DOI: https://doi.org/10.1093/rheumatology/kez328

Pinard J, Femia AN, Roman M, Alsarheed A, Joyce C, Lin J, Vleugels RA. Systemic Treatment for Clinically Amyopathic Dermatomyositis at 4 Tertiary Care Centers. JAMA Dermatol. 2019;155(4):494-6. DOI: https://doi.org/10.1001/jamadermatol.2018.5215

Cobos GA, Femia A, Vleugels RA. Dermatomyositis: An Update on Diagnosis and Treatment. Am J Clin Dermatol. 2020;21(3):339-53. DOI: https://doi.org/10.1007/s40257-020-00502-6

Wolstencroft PW, Chung L, Li S, Casciola-Rosen L, Fiorentino DF. Factors Associated With Clinical Remission of Skin Disease in Dermatomyositis. JAMA Dermatol. 2018;154(1):44-51. DOI: https://doi.org/10.1001/jamadermatol.2017.3758

Beckett M, Tan J, Bonnardeaux E, Dutz J, Shojania K, To F, et al. Tofacitinib therapy in refractory inflammatory myositis: a retrospective cohort study of 41 patients. Rheumatology (Oxford). 2023. DOI: https://doi.org/10.1093/rheumatology/kead404

Paik JJ, Lubin G, Gromatzky A, Mudd PN, Jr., Ponda MP, Christopher-Stine L. Use of Janus kinase inhibitors in dermatomyositis: a systematic literature review. Clin Exp Rheumatol. 2022. DOI: https://doi.org/10.55563/clinexprheumatol/hxin6o

Shneyderman M, Ahlawat S, Christopher-Stine L, Paik JJ. Calcinosis in refractory dermatomyositis improves with tofacitinib monotherapy: a case series. Rheumatology (Oxford). 2021;60(11):e387-e8. DOI: https://doi.org/10.1093/rheumatology/keab421

Wendel S, Venhoff N, Frye BC, May AM, Agarwal P, Rizzi M, et al. Successful treatment of extensive calcifications and acute pulmonary involvement in dermatomyositis with the Janus-Kinase inhibitor tofacitinib - A report of two cases. J Autoimmun. 2019;100:131-6. DOI: https://doi.org/10.1016/j.jaut.2019.03.003

Werth VP, Aggarwal R, Charles-Schoeman C, Schessl J, Levine T, Kopasz N, et al. Efficacy of intravenous immunoglobulins (IVIg) in improving skin symptoms in patients with dermatomyositis: a post-hoc analysis of the ProDERM study. EClinicalMedicine. 2023;64:102234. DOI: https://doi.org/10.1016/j.eclinm.2023.102234

Balin SJ, Wetter DA, Andersen LK, Davis MD. Calcinosis cutis occurring in association with autoimmune connective tissue disease: the Mayo Clinic experience with 78 patients, 1996-2009. Arch Dermatol. 2012;148(4):455-62. DOI: https://doi.org/10.1001/archdermatol.2011.2052

Jiang W, Yang H, Li S, Tian X, Wang G. Clinical features, treatments and outcomes of calcinosis in adult patients with dermatomyositis: a single cohort study. Rheumatology (Oxford). 2021;60(6):2958-62. DOI: https://doi.org/10.1093/rheumatology/keaa618

Traineau H, Aggarwal R, Monfort JB, Senet P, Oddis CV, Chizzolini C, et al. Treatment of calcinosis cutis in systemic sclerosis and dermatomyositis: A review of the literature. J Am Acad Dermatol. 2020;82(2):317-25. DOI: https://doi.org/10.1016/j.jaad.2019.07.006

Aggarwal R, Loganathan P, Koontz D, Qi Z, Reed AM, Oddis CV. Cutaneous improvement in refractory adult and juvenile dermatomyositis after treatment with rituximab. Rheumatology (Oxford). 2017;56(2):247-54. DOI: https://doi.org/10.1093/rheumatology/kew396

Chung L, Genovese MC, Fiorentino DF. A pilot trial of rituximab in the treatment of patients with dermatomyositis. Arch Dermatol. 2007;143(6):763-7. DOI: https://doi.org/10.1001/archderm.143.6.763

Alexanderson H, Bostrom C. Exercise therapy in patients with idiopathic inflammatory myopathies and systemic lupus erythematosus - A systematic literature review. Best Pract Res Clin Rheumatol. 2020;34(2):101547. DOI: https://doi.org/10.1016/j.berh.2020.101547

Pijnenburg L, Giannini M, Bouchard-Marmen M, Arnaud L, Barsotti S, Bellando-Randone S, et al. In inflammatory myopathies, dropped head/bent spine syndrome is associated with scleromyositis: an international case-control study. RMD Open. 2023;9(3). DOI: https://doi.org/10.1136/rmdopen-2023-003081corr1

Kleefeld F, Uruha A, Schanzer A, Nishimura A, Roos A, Schneider U, et al. Morphologic and Molecular Patterns of Polymyositis With Mitochondrial Pathology and Inclusion Body Myositis. Neurology. 2022;99(20):e2212-e22. DOI: https://doi.org/10.1212/WNL.0000000000201103

Temiz P, Weihl CC, Pestronk A. Inflammatory myopathies with mitochondrial pathology and protein aggregates. J Neurol Sci. 2009;278(1-2):25-9. DOI: https://doi.org/10.1016/j.jns.2008.11.010

Naddaf E. Inclusion body myositis: Update on the diagnostic and therapeutic landscape. Front Neurol. 2022;13:1020113. DOI: https://doi.org/10.3389/fneur.2022.1020113

Johnson C, Pinal-Fernandez I, Parikh R, Paik J, Albayda J, Mammen AL, et al. Assessment of Mortality in Autoimmune Myositis With and Without Associated Interstitial Lung Disease. Lung. 2016;194(5):733-7. DOI: https://doi.org/10.1007/s00408-016-9896-x

Hallowell RW, Danoff SK. Diagnosis and Management of Myositis-Associated Lung Disease. Chest. 2023;163(6):1476-91. DOI: https://doi.org/10.1016/j.chest.2023.01.031

Huang K, Vinik O, Shojania K, Yeung J, Shupak R, Nimmo M, Avina-Zubieta JA. Clinical spectrum andtherapeutics in Canadian patients with anti-melanoma differentiation-associated gene 5 (MDA5)-positive dermatomyositis: a case-based review. Rheumatol Int. 2019;39(11):1971-81. DOI: https://doi.org/10.1007/s00296-019-04398-2

Johnson SR. American College of Rheumatology (ACR) Guideline for the Screening, Monitoring, and Treatment of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Disease 2023 Available from: https://assets.contentstack.io/v3/assets/bltee37abb6b278ab2c/bltaedebda97a351d47/interstitial-lung-disease-guideline-summarytreatment-2023.pdf.

Aggarwal R, Lundberg IE, Song YW, Shaibani A, Werth VP, Maldonado MA. POS0839 RANDOMIZED, DOUBLE-BLIND, PLACEBO-CONTROLLED TRIAL TO EVALUATE EFFICACY AND SAFETY OF SCABATACEPT IN ADULTS WITH ACTIVE IDIOPATHIC INFLAMMATORY MYOPATHY. Annals of the Rheumatic Diseases. 2022;81(Suppl 1):711-. DOI: https://doi.org/10.1136/annrheumdis-2022-eular.7

Oddis CV, Rockette HE, Zhu L, Koontz DC, Lacomis D, Venturupalli S, et al. Randomized Trial of Tocilizumab in the Treatment of Refractory Adult Polymyositis and Dermatomyositis. ACR Open Rheumatol. 2022;4(11):983-90. DOI: https://doi.org/10.1002/acr2.11493

Beckett M, Dutz J, Huang K. Upadacitinib therapy in refractory inflammatory myositis: a case series of 10 patients. RMD Open. 2024;10(1). DOI: https://doi.org/10.1136/rmdopen-2023-003837

Chen Z, Wang X, Ye S. Tofacitinib in Amyopathic Dermatomyositis-Associated Interstitial Lung Disease. N Engl J Med. 2019;381(3):291-3. DOI: https://doi.org/10.1056/NEJMc1900045

Anolik JH, Barnard J, Owen T, Zheng B, Kemshetti S, Looney RJ, Sanz I. Delayed memory B cell recovery in peripheral blood and lymphoid tissue in systemic lupus erythematosus after B cell depletion therapy. Arthritis Rheum. 2007;56(9):3044-56. DOI: https://doi.org/10.1002/art.22810

Roccatello D, Fenoglio R, Caniggia I, Kamgaing J, Naretto C, Cecchi I, et al. Daratumumab monotherapy for refractory lupus nephritis. Nat Med. 2023;29(8):2041-7. DOI: https://doi.org/10.1038/s41591-023-02479-1

Landon-Cardinal O, Allard-Chamard H, Chapdelaine H, Doucet S, Rich E, Bourre-Tessier J. Daratumumab as a rescue therapy in severe refractory anti-SRP immunemediated necrotising myopathy. Ann Rheum Dis. 2023;82(4):579-80. DOI: https://doi.org/10.1136/ard-2022-223541

Holzer MT, Nies JF, Oqueka T, Huber TB, Kotter I, Krusche M. Successful Rescue Therapy With Daratumumab in Rapidly Progressive Interstitial Lung Disease Caused by MDA5-Positive Dermatomyositis. Chest. 2023;163(1):e1-e5. DOI: https://doi.org/10.1016/j.chest.2022.08.2209

Scheibe F, Ostendorf L, Pruss H, Radbruch H, Aschman T, Hoffmann S, et al. Daratumumab for treatmentrefractory antibody-mediated diseases in neurology. Eur J Neurol. 2022;29(6):1847-54. DOI: https://doi.org/10.1111/ene.15266

Schett G, Mackensen A, Mougiakakos D. CAR T-cell therapy in autoimmune diseases. Lancet. 2023;402(10416):2034-44. DOI: https://doi.org/10.1016/S0140-6736(23)01126-1

Taubmann J, Knitza J, Muller F, Volkl S, Aigner M, Kleyer A, et al. Rescue therapy of antisynthetase syndrome with CD19-targeted CAR-T-cells after failure of several B cell depleting antibodies. Rheumatology (Oxford). 2023. DOI: https://doi.org/10.1093/rheumatology/kead669

Pecher AC, Hensen L, Klein R, Schairer R, Lutz K, Atar D, et al. CD19-Targeting CAR T Cells for Myositis and Interstitial Lung Disease Associated With Antisynthetase Syndrome. JAMA. 2023;329(24):2154-62. DOI: https://doi.org/10.1001/jama.2023.8753

Muller F, Boeltz S, Knitza J, Aigner M, Volkl S, Kharboutli S, et al. CD19-targeted CAR T cells in refractory antisynthetase syndrome. Lancet. 2023;401(10379):815-8. DOI: https://doi.org/10.1016/S0140-6736(23)00023-5

Downloads

Published

2024-04-23

How to Cite

1.
Huang K, Pye A. Updates in Treatment of Refractory Inflammatory Myositis. Can Rheumatol Today [Internet]. 2024 Apr. 23 [cited 2024 Nov. 23];1(1):19–26. Available from: https://canadianrheumatologytoday.com/article/view/1-1-Huang_et_al

Issue

Volume 1, Issue 1, Spring 2024

Section

Articles

License

Copyright (c) 2024 Canadian Rheumatology Today

Creative Commons License

This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.