Idiopathic inflammatory myopathies - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

Idiopathic inflammatory myopathies (IIMs) constitute a heterogeneous group of systemic autoimmune disorders that cause inflammation of skeletal muscles. Inflammation often results in muscle weakness, and may involve skin, joints, lungs, and heart.
Certain subtypes of IIMs are associated with cancer occurrence, especially during the 3 years before and after IIM diagnosis.
Presentation of IIM is subacute or chronic, and rarely acute. Patients with subacute disease present with typical dermatomyositis rash without skeletal muscle involvement (DM sine myopathy). Severe presentations include generalized weakness, dysphagia, respiratory failure, and cardiac involvement.
Diagnosis is confirmed with a thorough history and physical exam, followed by corroborative investigations which include serum muscle-derived enzymes and damage markers, myositis-specific and myositis-associated autoantibodies, electromyography, magnetic resonance imaging and biopsy of muscles.
Remission induction of IIM (except inclusion body myositis) is usually achieved with corticosteroids and early introduction of corticosteroid-sparing immunosuppressants. Intravenous immunoglobulin is an effective adjunct therapy and can be considered in patients with refractory IIM.

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Definition

Epidemiology

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Citations

Key Articles

Lundberg IE, Fujimoto M, Vencovsky J, et al. Idiopathic inflammatory myopathies. Nat Rev Dis Primers. 2021 Dec 2;7(1):86.[Abstract]
Schmidt J. Current classification and management of inflammatory myopathies. J Neuromuscul Dis. 2018;5(2):109-29.[Abstract][Full Text]
Oldroyd A, Lilleker J, Chinoy H. Idiopathic inflammatory myopathies: a guide to subtypes, diagnostic approach and treatment. Clin Med (Lond). 2017 Jul;17(4):322-8.[Abstract][Full Text]
Oldroyd AGS, Lilleker JB, Amin T, et al. British Society for Rheumatology guideline on management of paediatric, adolescent and adult patients with idiopathic inflammatory myopathy. Rheumatology (Oxford). 2022 May 5;61(5):1760-8.[Abstract][Full Text]

Other Online Resources

Classification criteria for idiopathic inflammatory myopathies

Referenced Articles

1. Khoo T, Lilleker JB, Thong BY, et al. Epidemiology of the idiopathic inflammatory myopathies. Nat Rev Rheumatol. 2023 Nov;19(11):695-712.[Abstract]
2. Ashton C, Paramalingam S, Stevenson B, et al. Idiopathic inflammatory myopathies: a review. Intern Med J. 2021 Jun;51(6):845-52.[Abstract][Full Text]
3. Lundberg IE, Tjärnlund A, Bottai M, et al. 2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Ann Rheum Dis. 2017 Dec;76(12):1955-64.[Abstract][Full Text]
4. Lundberg IE, Fujimoto M, Vencovsky J, et al. Idiopathic inflammatory myopathies. Nat Rev Dis Primers. 2021 Dec 2;7(1):86.[Abstract]
5. Okiyama N. Clinical features and cutaneous manifestations of juvenile and adult patients of dermatomyositis associated with myositis-specific autoantibodies. J Clin Med. 2021 Apr 16;10(8):1725.[Abstract][Full Text]
6. McHugh NJ, Tansley SL. Autoantibodies in myositis. Nat Rev Rheumatol. 2018 Apr 20;14(5):290-302.[Abstract]
7. Shah M, Shinjo SK, Day J, et al. Cardiovascular manifestations in idiopathic inflammatory myopathies. Clin Rheumatol. 2023 Oct;42(10):2557-75.[Abstract][Full Text]
8. Oddis CV, Conte CG, Steen VD, et al. Incidence of polymyositis-dermatomyositis: a 20-year study of hospital diagnosed cases in Allegheny County, PA 1963-1982. J Rheumatol. 1990;17:1329-1334.[Abstract]
9. Medsger TA, Dawson WN Jr, Masi AT. The epidemiology of polymyositis. Am J Med. 1970;48:715-723.[Abstract]
10. Christopher-Stine L, Plotz PH. Adult inflammatory myopathies. Best Pract Res Clin Rheumatol. 2004;18:331-344.[Abstract]
11. Dorph C, Lundberg IE. Idiopathic inflammatory myopathies - myositis. Best Pract Res Clin Rheumatol. 2002;16:817-832.[Abstract]
12. Vivekanandam V, Bugiardini E, Merve A, et al. Differential diagnoses of inclusion body myositis. Neurol Clin. 2020 Aug;38(3):697-710.[Abstract]
13. Oflazer PS, Deymeer F, Parman Y. Sporadic-inclusion body myositis (s-IBM) is not so prevalent in Istanbul/Turkey: a muscle biopsy based survey. Acta Myol. 2011 Jun;30(1):34-6.[Abstract][Full Text]
14. Shelly S, Mielke MM, Mandrekar J, et al. Epidemiology and natural history of inclusion body myositis: a 40-year population-based study. Neurology. 2021 May 25;96(21):e2653-61.[Abstract]
15. Day J, Otto S, Proudman S, et al. Dysregulated innate immune function in the aetiopathogenesis of idiopathic inflammatory myopathies. Autoimmun Rev. 2017 Jan;16(1):87-95.[Abstract]
16. Dalakas MC. Pathogenesis and therapies of immune-mediated myopathies. Autoimmun Rev. 2012 Jan;11(3):203-6.[Abstract]
17. Moran EM, Mastaglia FL. Cytokines in immune-mediated inflammatory myopathies: cellular sources, multiple actions and therapeutic implications. Clin Exp Immunol. 2014 Dec;178(3):405-15.[Abstract][Full Text]
18. Dalakas MC. Inflammatory muscle diseases. N Engl J Med. 2015 Apr 30;372(18):1734-47.[Abstract]
19. Schiffenbauer A, Faghihi-Kashani S, O'Hanlon TP, et al. The effect of cigarette smoking on the clinical and serological phenotypes of polymyositis and dermatomyositis. Semin Arthritis Rheum. 2018 Dec;48(3):504-12.[Abstract][Full Text]
20. Carpenter S, Karpati G. Pathology of skeletal muscle. 2nd ed. New York, NY: Oxford University Press USA; 2001.
21. Dalakas MC. Inflammatory, immune, and viral aspects of inclusion-body myositis. Neurology. 2006;66(2 suppl 1):S33-S38.[Abstract]
22. Chou SM. Inclusion body myositis: a chronic persistent mumps myositis? Hum Pathol. 1986;17:765-777.[Abstract]
23. Dalakas MC, Pezeshkpour GH, Gravell M, et al. Polymyositis associated with AIDS retrovirus. JAMA. 1986 Nov 7;256(17):2381-3.[Abstract]
24. Dalakas MC, Rakocevic G, Shatunov A, et al. Inclusion body myositis with human immunodeficiency virus infection: four cases with clonal expansion of viral-specific T cells. Ann Neurol. 2007;61:466-475.[Abstract]
25. Saud A, Naveen R, Aggarwal R, et al. COVID-19 and myositis: what we know so far. Curr Rheumatol Rep. 2021 Jul 3;23(8):63.[Abstract][Full Text]
26. Kharouf F, Kenig A, Bohbot E, et al. Increased rates of idiopathic inflammatory myopathies during the COVID-19 pandemic: a single-centre experience. Clin Exp Rheumatol. 2023 Mar;41(2):316-21.[Abstract]
27. Karpati G, Hilton-Jones D, Griggs RC. Disorders of voluntary muscle. 7th ed. Cambridge, UK: Cambridge University Press; 2001.
28. Reed AM, Ytterberg SR. Genetic and environmental risk factors for idiopathic inflammatory myopathies. Rheum Dis Clin North Am. 2002 Nov;28(4):891-916.[Abstract]
29. Caughey GE, Gabb GM, Ronson S, et al. Association of statin exposure with histologically confirmed idiopathic inflammatory myositis in an Australian population. JAMA Intern Med. 2018 Sep 1;178(9):1224-9.[Abstract][Full Text]
30. Seidler AM, Gottlieb AB. Dermatomyositis induced by drug therapy: a review of case reports. J Am Acad Dermatol. 2008;59:872-880.[Abstract]
31. Close RM, Close LM, Galdun P, et al. Potential implications of six American Indian patients with myopathy, statin exposure and anti-HMGCR antibodies. Rheumatology (Oxford). 2021 Feb 1;60(2):692-8.[Abstract][Full Text]
32. Basharat P, Lahouti AH, Paik JJ, et al. Statin-induced anti-HMGCR-associated myopathy. J Am Coll Cardiol. 2016 Jul 12;68(2):234-5.[Abstract][Full Text]
33. Hamada N, Maeda A, Takase-Minegishi K, et al. Incidence and distinct features of immune checkpoint inhibitor-related myositis from idiopathic inflammatory myositis: a single-center experience with systematic literature review and meta-analysis. Front Immunol. 2021;12:803410.[Abstract][Full Text]
34. Opinc-Rosiak AH, Makowska JS. Environmental exposures as risk factors for idiopathic inflammatory myopathies. J Autoimmun. 2023 Nov;140:103095.[Abstract][Full Text]
35. Shelly S, Triplett JD, Pinto MV, et al. Immune checkpoint inhibitor-associated myopathy: a clinicoseropathologically distinct myopathy. Brain Commun. 2020;2(2):fcaa181.[Abstract][Full Text]
36. Monaghan M, Loh C, Jones S, et al. Inflammatory myositis secondary to anti-retroviral therapy in a child; case Rreport and review of the literature. J Neuromuscul Dis. 2021;8(6):1089-95.[Abstract]
37. Syrmou V, Liaskos C, Ntavari N, et al. COVID-19 vaccine-associated myositis: a comprehensive review of the literature driven by a case report. Immunol Res. 2023 Aug;71(4):537-46.[Abstract][Full Text]
38. Ferri C, Colaci M, Manzini CU, et al. Polymyositis following Pandemic Influenza A (H1N1) and 2009-10 Seasonal Trivalent Vaccines. Case Rep Rheumatol. 2012;2012:836930.[Abstract][Full Text]
39. Altman A, Szyper-Kravitz M, Shoenfeld Y. HBV vaccine and dermatomyositis: is there an association? Rheumatol Int. 2008 Apr;28(6):609-12.[Abstract]
40. Chinoy H, Adimulam S, Marriage F, et al. Interaction of HLA-DRB1*03 and smoking for the development of anti-Jo-1 antibodies in adult idiopathic inflammatory myopathies: a European-wide case study. Ann Rheum Dis. 2012 Jun;71(6):961-5.[Abstract][Full Text]
41. Okada S, Weatherhead E, Targoff IN, et al. Global surface ultraviolet radiation intensity may modulate the clinical and immunologic expression of autoimmune muscle disease. Arthritis Rheum. 2003 Aug;48(8):2285-93.[Abstract][Full Text]
42. Limaye V, Blumbergs P, Scott G et al. The epidemiology of dermatomyositis in South Australia. APLAR J. Rheumatol. 2007 May 15;10(2):94-100.
43. Svensson J, Arkema EV, Lundberg IE, et al. Incidence and prevalence of idiopathic inflammatory myopathies in Sweden: a nationwide population-based study. Rheumatology (Oxford). 2017 May 1;56(5):802-10.[Abstract][Full Text]
44. O'Hanlon T, Koneru B, Bayat E, et al. Immunogenetic differences between Caucasian women with and those without silicone implants in whom myositis develops. Arthritis Rheum. 2004 Nov;50(11):3646-50.[Abstract][Full Text]
45. Selva-O'Callaghan A, Tura JM, Grau-Junyent JM, et al. Silicone gel filled breast implants and dermatomyositis. Clin Exp Rheumatol. 2004 May-Jun;22(3):376.[Abstract]
46. Limaye S, Limaye V. Clinical characteristics of myositis associated with graft-versus-host disease. Curr Rheumatol Rep. 2021 Apr 24;23(5):30.[Abstract]
47. Reed AM, McNallan K, Wettstein P, et al. Does HLA-dependent chimerism underlie the pathogenesis of juvenile dermatomyositis? J Immunol. 2004 Apr 15;172(8):5041-6.[Abstract][Full Text]
48. Chanbour H, Jiblawi A, Aboudalle A, et al. Association of silicosis and dermatomyositis: case report and literature review. Cureus. 2021 Nov;13(11):e19875.[Abstract][Full Text]
49. Christopher-Stine L, Plotz PH. Myositis: an update on pathogenesis. Curr Opin Rheumatol. 2004 Nov;16(6):700-6.[Abstract]
50. Rothwell S, Cooper RG, Lundberg IE, et al. Dense genotyping of immune-related loci in idiopathic inflammatory myopathies confirms HLA alleles as the strongest genetic risk factor and suggests different genetic background for major clinical subgroups. Ann Rheum Dis. 2016 Aug;75(8):1558-66.[Abstract]
51. Rothwell S, Chinoy H, Lamb JA, et al. Focused HLA analysis in Caucasians with myositis identifies significant associations with autoantibody subgroups. Ann Rheum Dis. 2019 Jul;78(7):996-1002.[Abstract][Full Text]
52. Rothwell S, Cooper RG, Lundberg IE, et al. Immune-array analysis in sporadic inclusion body myositis reveals HLA-DRB1 amino acid heterogeneity across the myositis spectrum. Arthritis Rheumatol. 2017 May;69(5):1090-9.[Abstract][Full Text]
53. Lamb JA. The genetics of autoimmune myositis. Front Immunol. 2022;13:886290.[Abstract][Full Text]
54. Rothwell S, Amos CI, Miller FW, et al. Identification of novel associations and localization of signals in idiopathic inflammatory myopathies using genome-wide imputation. Arthritis Rheumatol. 2023 Jun;75(6):1021-7.[Abstract][Full Text]
55. Kochi Y, Kamatani Y, Kondo Y, et al. Splicing variant of WDFY4 augments MDA5 signalling and the risk of clinically amyopathic dermatomyositis. Ann Rheum Dis. 2018 Apr;77(4):602-11.[Abstract]
56. Chan SH, Bylstra Y, Teo JX, et al. Analysis of clinically relevant variants from ancestrally diverse Asian genomes. Nat Commun. 2022 Nov 5;13(1):6694.[Abstract][Full Text]
57. Dourado E, Bottazzi F, Cardelli C, et al. Idiopathic inflammatory myopathies: one year in review 2022. Clin Exp Rheumatol. 2023 Mar;41(2):199-213.[Abstract]
58. Lundberg IE, Miller FW, Tjärnlund A, et al. Diagnosis and classification of idiopathic inflammatory myopathies. J Intern Med. 2016 Jul;280(1):39-51.[Abstract][Full Text]
59. Banker BQ. Dermatomyostis of childhood, ultrastructural alterations of muscle and intramuscular blood vessels. J Neuropathol Exp Neurol. 1975;34:46-75.[Abstract]
60. Carpenter S, Karpati G, Rothman S, et al. The childhood type of dermatomyositis. Neurology. 1976;26:952-962.[Abstract]
61. Dalakas MC. Inflammatory disorders of muscle: progress in polymyositis, dermatomyositis and inclusion body myositis. Curr Opin Neurol. 2004;17:561-567.[Abstract]
62. Dalakas MC. Polymyositis, dermatomyositis and inclusion-body myositis. N Engl J Med. 1991 Nov 21;325(21):1487-98.[Abstract]
63. Choi YC, Dalakas MC. Expression of matrix metalloproteinases in the muscle of patients with inflammatory myopathies. Neurology. 2000;54:65-71.[Abstract]
64. Lindvall B, Dahlbom K, Henriksson KG, et al. The expression of adhesion molecules in muscle biopsies: the LFA-1/VLA-4 ratio in polymyositis. Acta Neurol Scand. 2003;107:134-141.[Abstract]
65. Snedden AM, Kellett KAB, Lilleker JB, et al. The role of protein aggregation in the pathogenesis of inclusion body myositis. Clin Exp Rheumatol. 2022 Feb;40(2):414-24.[Abstract]
66. Dalakas MC. Sporadic inclusion body myositis--diagnosis, pathogenesis and therapeutic strategies. Nat Clin Pract Neurol. 2006 Aug;2(8):437-47.[Abstract]
67. Rönnblom L, Eloranta ML. The interferon signature in autoimmune diseases. Curr Opin Rheumatol. 2013 Mar;25(2):248-53.[Abstract]
68. Arshanapalli A, Shah M, Veerula V, et al. The role of type I interferons and other cytokines in dermatomyositis. Cytokine. 2015 Jun;73(2):319-25.[Abstract]
69. Zampieri S, Ghirardello A, Iaccarino L, et al. Polymyositis-dermatomyositis and infections. Autoimmunity. 2006 May;39(3):191-6.[Abstract]
70. Kawai T, Akira S. The role of pattern-recognition receptors in innate immunity: update on Toll-like receptors. Nat Immunol. 2010 May;11(5):373-84.[Abstract][Full Text]
71. Baccala R, Hoebe K, Kono DH, et al. TLR-dependent and TLR-independent pathways of type I interferon induction in systemic autoimmunity. Nat Med. 2007 May;13(5):543-51.[Abstract]
72. Schmidt J. Current classification and management of inflammatory myopathies. J Neuromuscul Dis. 2018;5(2):109-29.[Abstract][Full Text]
73. Preuße C, Goebel HH, Held J, et al. Immune-mediated necrotizing myopathy is characterized by a specific Th1-M1 polarized immune profile. Am J Pathol. 2012 Dec;181(6):2161-71.[Abstract][Full Text]
74. Oldroyd AGS, Callen JP, Chinoy H, 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 Dec;19(12):805-17.[Abstract][Full Text]
75. 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 Apr;62(4):465-72.[Abstract][Full Text]
76. Medical Research Council. Aids to the examination of the peripheral nervous system. 1976 [internet publication].[Full Text]
77. Oldroyd A, Lilleker J, Chinoy H. Idiopathic inflammatory myopathies: a guide to subtypes, diagnostic approach and treatment. Clin Med (Lond). 2017 Jul;17(4):322-8.[Abstract][Full Text]
78. Lundberg I. Polymyositis and dermatomyositis. In: Bijlsma J, ed. Eular textbook on rheumatic diseases. 1st ed. London: BMJ Group; 2012: 568-93.
79. Lu X, Peng Q, Wang G. Anti-MDA5 antibody-positive dermatomyositis: pathogenesis and clinical progress. Nat Rev Rheumatol. 2024 Jan;20(1):48-62.[Abstract]
80. Chua CG, Low JZ, Lim WY, et al. Characteristics of anti-transcriptional intermediary factor 1 gamma autoantibody-positive dermatomyositis patients in Singapore. Ann Acad Med Singap. 2022 Dec;51(12):755-65.[Abstract][Full Text]
81. Dion E, Chérin P. Use of muscular MRI in inflammatory myopathies [in French]. Rev Med Interne. 2004;25:435-441.[Abstract]
82. Johnson SR, Bernstein EJ, Bolster MB, et al. 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guideline for the screening and monitoring of interstitial lung dsease in people with systemic autoimmune rheumatic diseases. Arthritis Care Res (Hoboken). 2024 Aug;76(8):1070-82.[Abstract][Full Text]
83. Lotz BP, Engel AG, Nishino H, et al. Inclusion body myositis. Observations in 40 patients. Brain. 1989;112:727-747.[Abstract]
84. Badrising UA, Maat-Schieman ML, van Houwelingen JC, et al. Inclusion body myositis. Clinical features and clinical course of the disease in 64 patients. J Neurol. 2005;252:1448-1454.[Abstract]
85. Betteridge Z, McHugh N. Myositis-specific autoantibodies: an important tool to support diagnosis of myositis. J Intern Med. 2016 Jul;280(1):8-23.[Abstract][Full Text]
86. Motomura K, Yamashita H, Yamada S, et al. Clinical characteristics and prognosis of polymyositis and dermatomyositis associated with malignancy: a 25-year retrospective study. Rheumatol Int. 2019 Oct;39(10):1733-9.[Abstract]
87. Engel A, Franzini-Armstrong C. Myology: basic and clinical. 3rd ed. New York: McGraw-Hill, Medical Publishing Division; 2004.
88. Al-Nahhas A, Jawad AS. PET/CT imaging in inflammatory myopathies. Ann N Y Acad Sci. 2011 Jun;1228:39-45.[Abstract]
89. Fan X, Rouleau GA. Progress in understanding the pathogenesis of oculopharyngeal muscular dystrophy. Can J Neurol Sci. 2003;30:8-14.[Abstract]
90. Rose MR; ENMC IBM Working Group. 188th ENMC international workshop: inclusion body myositis, 2-4 December 2011, Naarden, the Netherlands. Neuromuscul Disord. 2013 Dec;23(12):1044-55.[Abstract][Full Text]
91. The Myositis Association. Diagnostic criteria for necrotizing myopathy [internet publication].[Full Text]
92. Colak A, Benli K, Dönmez T, et al. Papillary carcinoma of the sphenoid sinus associated with sphenoid sinus abscess presenting as cavernous sinus syndrome. A case report. J Clin Neuroophthalmol. 1990 Mar;10(1):18-20.[Abstract]
93. Oldroyd AGS, Lilleker JB, Amin T, et al. British Society for Rheumatology guideline on management of paediatric, adolescent and adult patients with idiopathic inflammatory myopathy. Rheumatology (Oxford). 2022 May 5;61(5):1760-8.[Abstract][Full Text]
94. Johnson SR, Bernstein EJ, Bolster MB, et al. 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) Guideline for the treatment of interstitial lung disease in people with systemic autoimmune rheumatic diseases. Arthritis Rheumatol. 2024 Aug;76(8):1182-200.[Abstract][Full Text]
95. Lim J, Eftimov F, Verhamme C, et al. Intravenous immunoglobulins as first-line treatment in idiopathic inflammatory myopathies: a pilot study. Rheumatology (Oxford). 2021 Apr 6;60(4):1784-92.[Abstract][Full Text]
96. Lee H, Chung SJ, Kim SH, et al. Treatment outcomes of infectious and non-infectious acute exacerbation of myositis-related interstitial lung disease. Front Med (Lausanne). 2021;8:801206.[Abstract][Full Text]
97. Wiala A, Vujic I, Richter L, et al. Dermatomyositis requires long-term treatment with combined immunosuppressive and immunoglobulin therapy. J Dtsch Dermatol Ges. 2021 Mar;19(3):456-8.[Abstract]
98. Hoff LS, de Souza FHC, Miossi R, et al. Long-term effects of early pulse methylprednisolone and intravenous immunoglobulin in patients with dermatomyositis and polymyositis. Rheumatology (Oxford). 2022 Apr 11;61(4):1579-88.[Abstract][Full Text]
99. Schneider-Gold C, Hartung HP, Gold R. Mycophenolate mofetil and tacrolimus: new therapeutic options in neuroimmunological diseases. Muscle Nerve. 2006;34:284-291.[Abstract]
100. Dalakas MC. Immunotherapy of myositis: issues, concerns and future prospects. Nat Rev Rheumatol. 2010;6:129-137.[Abstract]
101. Dalakas MC. Inflammatory myopathies: management of steroid resistance. Curr Opin Neurol. 2011 Oct;24(5):457-62.[Abstract]
102. Wiendl H. Idiopathic inflammatory myopathies: current and future therapeutic options. Neurotherapeutics. 2008;5:548-557.[Abstract]
103. 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;65:314-324.[Abstract]
104. Fasano S, Gordon P, Hajji R, et al. Rituximab in the treatment of inflammatory myopathies: a review. Rheumatology (Oxford). 2017 Jan;56(1):26-36.[Abstract][Full Text]
105. Valiyil R, Casciola-Rosen L, Hong G, et al. Rituximab therapy for myopathy associated with anti-signal recognition particle antibodies: a case series. Arthritis Care Res (Hoboken). 2010 Sep;62(9):1328-34.[Abstract][Full Text]
106. Mok CC, Ho LY, To CH. Rituximab for refractory polymyositis: an open-label prospective study. J Rheumatol. 2007 Sep;34(9):1864-8.[Abstract]
107. Ge Y, Li S, Tian X, et al. Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis responds to rituximab therapy. Clin Rheumatol. 2021 Jun;40(6):2311-7.[Abstract]
108. Amato AA, Griggs RC. Treatment of idiopathic inflammatory myopathies. Curr Opin Neurol. 2003 Oct;16(5):569-75.[Abstract]
109. Tjärnlund A, Tang Q, Wick C, et al. Abatacept in the treatment of adult dermatomyositis and polymyositis: a randomised, phase IIb treatment delayed-start trial. Ann Rheum Dis. 2018 Jan;77(1):55-62.[Abstract]
110. Connolly CM, Gupta L, Fujimoto M, et al. Idiopathic inflammatory myopathies: current insights and future frontiers. Lancet Rheumatol. 2024 Feb;6(2):e115-27.[Abstract]
111. Paik JJ, Shneyderman M, Gutierrez-Alamillo L, et al. Long-term extension study of tofacitinib in refractory dermatomyositis. Arthritis Rheumatol. 2022 Feb;74(2):371-2.[Abstract]
112. ClinicalTrials.gov. Baricitinib in patients with relapsing or naïve dermatomyositis (BIRD). ClinicalTrials.gov Identifier: NCT04972760. Mar 2024 [internet publication].[Full Text]
113. ClinicalTrials.gov. Baricitinib in the treatment of new-onset juvenile dermatomyositis (MYOCIT) (MYOCIT). ClinicalTrials.gov Identifier: NCT05524311. Jan 2024 [internet publication].[Full Text]
114. Chen Z, Wang X, Ye S. Tofacitinib in amyopathic dermatomyositis-associated interstitial lung disease. N Engl J Med. 2019 Jul 18;381(3):291-3.[Abstract]
115. Shirai T, Machiyama T, Sato H, et al. Intensive induction therapy combining tofacitinib, rituximab and plasma exchange in severe anti-melanoma differentiation-associatedprotein-5 antibody-positive dermatomyositis. Clin Exp Rheumatol. 2023 Mar;41(2):291-300.[Abstract]
116. Fan L, Lyu W, Liu H, et al. A retrospective analysis of outcome in melanoma differentiation-associated gene 5-related interstitial lung disease treated with Ttofacitinib or tacrolimus. J Rheumatol. 2022 Dec;49(12):1356-64.[Abstract][Full Text]
117. Peter HH, Ochs HD, Cunningham-Rundles C, et al. Targeting FcRn for immunomodulation: benefits, risks, and practical considerations. J Allergy Clin Immunol. 2020 Sep;146(3):479-91.e5.[Abstract][Full Text]
118. ClinicalTrials.gov. A study of nipocalimab in participants with active idiopathic inflammatory myopathies (SPIREA). ClinicalTrials.gov Identifier: NCT05379634. Jul 2024 [internet publication].[Full Text]
119. ClinicalTrials.gov. A study to assess the long-term safety and efficacy of a subcutaneous formulation of efgartigimod in adults with active idiopathic inflammatory myopathy (ALKIVIA+). ClinicalTrials.gov Identifier: NCT05979441. Jul 2024 [internet publication].[Full Text]
120. ClinicalTrials.gov. A study to investigate the efficacy and safety of efgartigimod PH20 SC in adult participants with active idiopathic inflammatory myopathy. (ALKIVIA). ClinicalTrials.gov Identifier: NCT05523167. Jul 2024 [internet publication].[Full Text]
121. Wilfong EM, Aggarwal R. Role of antifibrotics in the management of idiopathic inflammatory myopathy associated interstitial lung disease. Ther Adv Musculoskelet Dis. 2021;13:1759720X211060907.[Abstract][Full Text]
122. Li T, Guo L, Chen Z, et al. Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis. Sci Rep. 2016 Sep 12;6:33226.[Abstract][Full Text]
123. Liang J, Cao H, Yang Y, et al. Efficacy and tolerability of nintedanib in idiopathic-inflammatory-myopathy-related interstitial lung disease: a pilot study. Front Med (Lausanne). 2021;8:626953.[Abstract][Full Text]
124. Goel N, Needham M, Soler-Ferran D, et al. POS1342 Depletion of KLRG1+ T cells in a first-in-human clinical trial of ABC008 in inclusion body myositis (IBM). Nature. 2017 Oct 19;550(7676):407-10.
125. Benveniste O, Hogrel JY, Belin L, et al. Sirolimus for treatment of patients with inclusion body myositis: a randomised, double-blind, placebo-controlled, proof-of-concept, phase 2b trial. Lancet Rheumatol. 2021 Jan;3(1):e40-8.[Abstract]
126. Lyu X, Gupta L, Tholouli E, et al. Chimeric antigen receptor T cell therapy: a new emerging landscape in autoimmune rheumatic diseases. Rheumatology (Oxford). 2024 May 2;63(5):1206-16.[Abstract][Full Text]
127. Schett G, Mackensen A, Mougiakakos D. CAR T-cell therapy in autoimmune diseases. Lancet. 2023 Nov 25;402(10416):2034-44.[Abstract]
128. ClinicalTrials.gov. IMPT-514 in systemic lupus erythematosus, anca-associated vasculitis, and idiopathic inflammatory myopathy. ClinicalTrials.gov Identifier: NCT06462144. Jul 2024 [internet publication].[Full Text]
129. American College of Rheumatology. 2022 American College of Rheumatology (ACR) guideline for vaccinations in patients with rheumatic and musculoskeletal diseases. Feb 2023 [internet publication].[Full Text]
130. Feasby T, Banwell B, Benstead T, et al. Guidelines on the use of intravenous immune globulin for neurologic conditions. Transfus Med Rev. 2007;21(2 suppl 1):S57-S107.[Abstract]
131. Elovaara I, Apostolski S, van Doorn P, et al. EFNS guidelines for the use of intravenous immunoglobulin in treatment of neurological diseases: EFNS task force on the use of intravenous immunoglobulin in treatment of neurological diseases. Eur J Neurol. 2008 Sep;15(9):893-908.[Abstract][Full Text]
132. Dobloug GC, Svensson J, Lundberg IE, et al. Mortality in idiopathic inflammatory myopathy: results from a Swedish nationwide population-based cohort study. Ann Rheum Dis. 2018 Jan;77(1):40-7.[Abstract]
133. Sultan SM, Ioannou Y, Moss K, et al. Outcome in patients with idiopathic inflammatory myositis: morbidity and mortality. Rheumatology (Oxford). 2002;41:22-26.[Abstract][Full Text]
134. Marie I, Hachulla E, Hatron PY, et al. Polymyositis and dermatomyositis: short term and longterm outcome, and predictive factors of prognosis. J Rheumatol. 2001;28:2230-2237.[Abstract]
135. So J, So H, Wong VT, et al. Predictors of rapidly progressive interstitial lung disease and mortality in patients with autoantibodies against melanoma differentiation-associated protein 5 dermatomyositis. Rheumatology (Oxford). 2022 Nov 2;61(11):4437-44.[Abstract][Full Text]
136. Limaye V, Hakendorf P, Woodman RJ, et al. Mortality and its predominant causes in a large cohort of patients with biopsy-determined inflammatory myositis. Intern Med J. 2012 Feb;42(2):191-8.[Abstract][Full Text]
137. Briani C, Doria A, Sarzi-Puttini P, et al. Update on idiopathic inflammatory myopathies. Autoimmunity. 2006;39:161-170.[Abstract]
138. de Merieux P, Verity MA, Clements PJ, et al. Esophageal abnormalities and dysphagia in polymyositis and dermatomyositis. Arthritis Rheum. 1983 Aug;26(8):961-8.[Abstract]
139. Kagen LJ, Hochman RB, Strong EW. Cricopharyngeal obstruction in inflammatory myopathy (polymyositis/dermatomyositis). Report of three cases and review of the literature. Arthritis Rheum. 1985;28:630-636.[Abstract]
140. Madan V, Chinoy H, Griffiths CE, et al. Defining cancer risk in dermatomyositis: part I. Clin Exp Dermatol. 2009 Jun;34(4):451-5.[Abstract]
141. Buchbinder R, Forbes A, Hall S, et al. Incidence of malignant disease in biopsy-proven inflammatory myopathy: a population-based cohort study. Ann Intern Med. 2001 Jun 19;134(12):1087-95.[Abstract]
142. Wakata N, Kurihara T, Saito E, et al. Polymyositis and dermatomyositis associated with malignancy: a 30-year retrospective study. Int J Dermatol. 2002 Nov;41(11):729-34.[Abstract]
143. Hill CL, Zhang Y, Sigurgeirsson B, et al. Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. Lancet. 2001 Jan 13;357(9250):96-100.[Abstract]
144. Callen JP. Relation between dermatomyositis and polymyositis and cancer. Lancet. 2001 Jan 13;357(9250):85-6.[Abstract]
145. Greenberg SA, Amato AA. Inflammatory myopathy associated with mixed connective tissue disease and scleroderma renal crisis. Muscle Nerve. 2001 Nov;24(11):1562-6.[Abstract]