{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T13:37:27Z","timestamp":1760189847098,"version":"3.28.0"},"reference-count":71,"publisher":"Rambam Health Corporation","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Rambam Maimonides Med J"],"abstract":"<jats:p>Idiopathic inflammatory myopathies (IIM) are a rare group of disorders that feature progressive immune-mediated skeletal muscle destruction along with skin, lung, and joint involvement. Management of IIMs necessitates glucocorticoid therapy followed by conventional steroid-sparing agents to control disease activity. In the settings of refractory myositis or life-threatening manifestations, e.g. lung involvement or oropharyngeal dysphagia, second-line therapies are needed to minimize disease burden, avoid end-organ damage and steroid toxicity, and decrease mortality. These therapies may include biological disease-modifying antirheumatic drugs (bDMARDs), and to a lesser extent, targeted synthetic disease-modifying antirheumatic drugs (TSD). This article reviews the current use of bDMARDs, e.g. intravenous immunoglobulin and rituximab, and a TSD\u2014Janus kinase inhibitors (JAKI)\u2014along with their indications, efficacy, and safety in managing IIM.<\/jats:p>","DOI":"10.5041\/rmmj.10495","type":"journal-article","created":{"date-parts":[[2023,4,30]],"date-time":"2023-04-30T11:00:51Z","timestamp":1682852451000},"page":"e0008","source":"Crossref","is-referenced-by-count":4,"title":["Biological Therapies in Inflammatory Myopathies"],"prefix":"10.5041","volume":"14","author":[{"name":"Rheumatology Unit, Meir Medical Center, Kfar Saba, Israel","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Abd El Haleem","family":"Natour","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"name":"Department of Internal Medicine A, Meir Medical Center, Kfar Saba, Israel","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shaye","family":"Kivity","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"name":"Rheumatology Unit, Meir Medical Center, Kfar Saba, Israel","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"2983","published-online":{"date-parts":[[2023,4,30]]},"reference":[{"key":"ref0","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1186\/s42358-018-0030-z","article-title":"Favorable rituximab response in patients with refractory idiopathic inflammatory myopathies","volume":"58","author":"de Souza","year":"2018","unstructured":"1. de Souza FHC, Miossi R, Moraes JCB, Bonf\u00e1 E, Shinjo SK. Favorable rituximab response in patients with refractory idiopathic inflammatory myopathies. Adv Rheumatol 2018;58:31.","journal-title":"Adv Rheumatol"},{"key":"ref1","doi-asserted-by":"publisher","first-page":"1729","DOI":"10.1172\/JCI117520","article-title":"High-dose intravenous immunoglobulin exerts its beneficial effect in patients with dermatomyositis by blocking endomysial deposition of activated complement fragments","volume":"94","author":"Basta","year":"1994","unstructured":"2. Basta M, Dalakas MC. High-dose intravenous immunoglobulin exerts its beneficial effect in patients with dermatomyositis by blocking endomysial deposition of activated complement fragments. J Clin Invest 1994;94:1729-35.","journal-title":"J Clin Invest"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"329","DOI":"10.1056\/NEJM198602063140601","article-title":"Microvascular deposition of complement membrane attack complex in dermatomyositis","volume":"314","author":"Kissel","year":"1986","unstructured":"3. Kissel JT, Mendell JR, Rammohan KW. Microvascular deposition of complement membrane attack complex in dermatomyositis. N Engl J Med 1986;314: 329-34.","journal-title":"N Engl J Med"},{"key":"ref3","doi-asserted-by":"publisher","first-page":"1887","DOI":"10.1093\/brain\/awh518","article-title":"Gene expression profile in the muscles of patients with inflammatory myopathies: effect of therapy with IVIg and biologic validation of clinical relevant genes","volume":"128","author":"Raju","year":"2005","unstructured":"4. Raju R, Dalakas MC. Gene expression profile in the muscles of patients with inflammatory myopathies: effect of therapy with IVIg and biologic validation of clinical relevant genes. Brain 2005;128:1887-96.","journal-title":"Brain"},{"key":"ref4","doi-asserted-by":"publisher","first-page":"484","DOI":"10.1126\/science.291.5503.484","article-title":"Anti-inflammatory activity of IVIG mediated through the inhibitory Fc receptor","volume":"291","author":"Samuelsson","year":"2001","unstructured":"5. Samuelsson A, Towers TL, Ravetch JV. Anti-inflammatory activity of IVIG mediated through the inhibitory Fc receptor. Science 2001;291:484-6.","journal-title":"Science"},{"key":"ref5","doi-asserted-by":"publisher","unstructured":"6. Dalakas MC. Mechanistic effects of IVIG in neuroinflammatory diseases: conclusions based on clinicopathologic correlations. 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Results and long-term followup of intravenous immunoglobulin infusions in chronic, refractory polymyositis: an open study with thirty-five adult patients. Arthritis Rheum 2002;46:467-74.","journal-title":"Arthritis Rheum"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"1264","DOI":"10.1056\/NEJMoa2117912","article-title":"Trial of intravenous immune globulin in dermatomyositis","volume":"387","author":"Aggarwal","year":"2022","unstructured":"9. Aggarwal R, Charles-Schoeman C, Schessl J, et al. Trial of intravenous immune globulin in dermatomyositis. N Engl J Med 2022;387:1264-78.","journal-title":"N Engl J Med"},{"key":"ref9","doi-asserted-by":"publisher","first-page":"1234","DOI":"10.1093\/rheumatology\/keaa443","article-title":"Long-term efficacy of adding intravenous immunoglobulins as treatment of refractory dysphagia related to myositis: a retrospective analysis","volume":"60","author":"Giannini","year":"2021","unstructured":"10. Giannini M, Fiorella ML, Tampoia M, et al. Long-term efficacy of adding intravenous immunoglobulins as treatment of refractory dysphagia related to myositis: a retrospective analysis. Rheumatology (Oxford) 2021;60:1234-42.","journal-title":"Rheumatology (Oxford)"},{"key":"ref10","doi-asserted-by":"publisher","first-page":"906","DOI":"10.1111\/ced.13607","article-title":"Intravenous immunoglobulin is an effective treatment for refractory cutaneous dermatomyositis","volume":"43","author":"Galimberti","year":"2018","unstructured":"11. Galimberti F, Kooistra L, Li Y, Chatterjee S, Fernandez AP. Intravenous immunoglobulin is an effective treatment for refractory cutaneous dermatomyositis. Clin Exp Dermatol 2018;43:906-12.","journal-title":"Clin Exp Dermatol"},{"key":"ref11","doi-asserted-by":"publisher","first-page":"102997","DOI":"10.1016\/j.autrev.2021.102997","article-title":"Efficacy and safety of intravenous and subcutaneous immunoglobulin therapy in idiopathic inflammatory myopathy: a systematic review and meta-analysis","volume":"21","author":"Goswami","year":"2022","unstructured":"12. Goswami RP, Haldar SN, Chatterjee M, et al. Efficacy and safety of intravenous and subcutaneous immunoglobulin therapy in idiopathic inflammatory myopathy: a systematic review and meta-analysis. Autoimmun Rev 2022;21:102997.","journal-title":"Autoimmun Rev"},{"key":"ref12","doi-asserted-by":"publisher","first-page":"157","DOI":"10.2147\/TCRM.S157085","article-title":"Increased risk of venous thromboembolism associated with polymyositis and dermatomyositis: a meta-analysis","volume":"14","author":"Li","year":"2018","unstructured":"13. Li Y, Wang P, Li L, Wang F, Liu Y. 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J Neurol Neurosurg Psychiatry 2022;93: 876-85.","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"ref16","doi-asserted-by":"publisher","first-page":"357","DOI":"10.1111\/j.1525-1470.2011.01501.x","article-title":"Juvenile dermatomyositis: immunopathogenesis, role of myositis-specific autoantibodies, and review of rituximab use","volume":"28","author":"YE","year":"2011","unstructured":"17. Chiu YE, Co DO. Juvenile dermatomyositis: immunopathogenesis, role of myositis-specific autoantibodies, and review of rituximab use. Pediatr Dermatol 2011; 28:357-67.","journal-title":"Pediatr Dermatol"},{"key":"ref17","doi-asserted-by":"publisher","first-page":"158","DOI":"10.1186\/s13075-018-1650-8","article-title":"Serum levels of B-cell activating factor of the TNF family (BAFF) correlate with anti-Jo-1 autoantibodies levels and disease activity in patients with anti-Jo-1positive polymyositis and dermatomyositis","volume":"20","author":"Kry\u0161t\u016ffkov\u00e1","year":"2018","unstructured":"18. Kry\u0161t\u016ffkov\u00e1 O, Hulejov\u00e1 H, Mann HF, et al. Serum levels of B-cell activating factor of the TNF family (BAFF) correlate with anti-Jo-1 autoantibodies levels and disease activity in patients with anti-Jo-1positive polymyositis and dermatomyositis. Arthritis Res Ther 2018;20:158.","journal-title":"Arthritis Res Ther"},{"key":"ref18","doi-asserted-by":"publisher","first-page":"314","DOI":"10.1002\/art.37754","article-title":"Rituximab in the treatment of refractory adult and juvenile dermatomyositis and adult polymyositis: a randomized, placebo-phase trial","volume":"65","author":"Oddis","year":"2013","unstructured":"19. Oddis CV, Reed AM, Aggarwal R, et al. Rituximab in the treatment of refractory adult and juvenile dermatomyositis and adult polymyositis: a randomized, placebo-phase trial. Arthritis Rheum 2013;65:314-24.","journal-title":"Arthritis Rheum"},{"key":"ref19","doi-asserted-by":"publisher","first-page":"1051609","DOI":"10.3389\/fimmu.2022.1051609","article-title":"Efficacy and safety of rituximab treatment in patients with idiopathic inflammatory myopathies: a systematic review and meta-analysis","volume":"13","author":"Zhen","year":"2022","unstructured":"20. Zhen C, Hou Y, Zhao B, Ma X, Dai T, Yan C. 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Rheumatology (Oxford) 2023;62:e16-17.","DOI":"10.1093\/rheumatology\/keac398"},{"key":"ref22","doi-asserted-by":"publisher","first-page":"1243","DOI":"10.1093\/rheumatology\/keac438","article-title":"Low-dose rituximab is efficacious in refractory idiopathic inflammatory myopathies","volume":"62","author":"Janardana","year":"2023","unstructured":"23. Janardana R, Amin SN, Rajasekhar L, et al. Low-dose rituximab is efficacious in refractory idiopathic inflammatory myopathies. Rheumatology (Oxford) 2023;62:1243-7.","journal-title":"Rheumatology (Oxford)"},{"key":"ref23","doi-asserted-by":"publisher","first-page":"105","DOI":"10.1016\/j.rmed.2020.105983","article-title":"Ultra-low dose rituximab as add-on therapy in anti-MDA5-positive patients with polymyositis\/dermatomyositis associated ILD","volume":"172","author":"Mao","year":"2020","unstructured":"24. Mao M, Xia S, Guo B, et al. Ultra-low dose rituximab as add-on therapy in anti-MDA5-positive patients with polymyositis\/dermatomyositis associated ILD. Respir Med 2020;172:105","journal-title":"Respir Med"},{"key":"ref24","doi-asserted-by":"publisher","first-page":"664","DOI":"10.1002\/ana.20464","article-title":"Interferon-\u03b1\/\u03b2-mediated innate immune mechanisms in dermatomyositis","volume":"57","author":"Greenberg","year":"2005","unstructured":"25. Greenberg SA, Pinkus JL, Pinkus GS, et al. Interferon-\u03b1\/\u03b2-mediated innate immune mechanisms in dermatomyositis. Ann Neurol 2005;57:664-78.","journal-title":"Ann Neurol"},{"key":"ref25","doi-asserted-by":"publisher","first-page":"195","DOI":"10.1002\/art.41096","article-title":"Circulating interferon\u2010\u03b1 measured with a highly sensitive assay as a biomarker for juvenile inflammatory myositis activity: comment on the article by Mathian et al","volume":"72","author":"Melki","year":"2020","unstructured":"26. Melki I, Devilliers H, Gitiaux C, et al. Circulating interferon\u2010\u03b1 measured with a highly sensitive assay as a biomarker for juvenile inflammatory myositis activity: comment on the article by Mathian et al. Arthritis Rheumatol 2020;72:195-7.","journal-title":"Arthritis Rheumatol"},{"key":"ref26","doi-asserted-by":"publisher","unstructured":"27. Ladislau L, Su\u00e1rez-Calvet X, Toquet S, et al. JAK inhibitor improves type I interferon induced damage: proof of concept in dermatomyositis. Brain 2018:141: 1609-21.","DOI":"10.1093\/brain\/awy105"},{"key":"ref27","doi-asserted-by":"publisher","first-page":"2537","DOI":"10.1056\/NEJMc1412997","article-title":"Remission of recalcitrant dermatomyositis treated with ruxolitinib","volume":"371","author":"Hornung","year":"2014","unstructured":"28. Hornung T, Janzen V, Heidgen F-J, Wolf D, Bieber T, Wenzel J. Remission of recalcitrant dermatomyositis treated with ruxolitinib. N Engl J Med 2014;371: 2537-8.","journal-title":"N Engl J Med"},{"key":"ref28","doi-asserted-by":"publisher","first-page":"406","DOI":"10.1136\/annrheumdis-2020-218690","article-title":"Janus kinase (JAK) inhibition with baricitinib in refractory juvenile dermatomyositis","volume":"80","author":"Kim","year":"2020","unstructured":"29. Kim H, Dill S, O'Brien M, et al. Janus kinase (JAK) inhibition with baricitinib in refractory juvenile dermatomyositis. Ann Rheum Diseases 2020;80:406-8.","journal-title":"Ann Rheum Diseases"},{"key":"ref29","doi-asserted-by":"publisher","first-page":"5801","DOI":"10.1093\/rheumatology\/keab116","article-title":"JAK inhibitors are effective in a subset of patients with juvenile dermatomyositis: a monocentric retrospective study","volume":"60","author":"Le Voyer","year":"2021","unstructured":"30. Le Voyer T, Gitiaux C, Authier F-J, et al. JAK inhibitors are effective in a subset of patients with juvenile dermatomyositis: a monocentric retrospective study. 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