Idiopathic Inflammatory Myopathy: A Hospital-based Case Review in the State of Perak, Malaysia.
DOI:
https://doi.org/10.70672/r4fzzr88Keywords:
Amyopathic dermatomyositis, clinical characteristics, dermatomyositis, EULAR/ACR criteria, idiopathic inflammatory myopathy, polymyositisAbstract
Background: Idiopathic inflammatory myopathy (IIM) is a rare condition characterized by proximal muscle weakness and myositis. It includes dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM), and juvenile dermatomyositis (JDM). They may share clinical and histological features but differ in underlying pathophysiology. Objective: To assess the diagnostic probabilities, and clinical characteristics of IIM. Methods: Clinical data from 50 IIM patients were retrospectively reviewed from Rheumatology and Dermatology clinics across four centres in Perak, Malaysia (2013–2023) and were evaluated using the 2017 The European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for adult and juvenile IIM. Results: According to the EULAR/ACR criteria, 98.2% of cases were classified as definite IIM (mean score 10.6), 74.1% as probable (mean score 6.6), and 47.8% as possible (mean score 6.4). IIM was excluded in 36% of patients (mean score 3.9; probability 20.8%). Eighty-four percent had DM, 8% had ADM or PM, with a mean age of 45.9±18.1 years. 95.6% were diagnosed within a year of symptom onset, and 72% showed proximal myopathy, significantly common in DM (P=0.001). Ethnic breakdown showed 46% Chinese, 42% Malay, and 8% Indian patients, with Malays developing DM at a younger age (39.8±16.9 years). The shawl sign and telangiectasia were more common in Chinese (P=0.018) and Malay (P=0.023) patients, respectively. Conclusion: The EULAR/ACR scoring system effectively identified IIM subgroups. Despite varying probabilities, cases of both DM and ADM were classified into definite, probable, and possible categories, with or without biopsy data. DM was more prevalent than ADM, primarily affecting Chinese patients, while Malays had earlier onset.
References
1. Mandel DE, Malemud CJ, Askari AD. Idiopathic inflammatory myopathies: A review of the classification and impact of pathogenesis. International journal of molecular sciences. 2017; 18(5):1084.
2. Callen JP. Cutaneous manifestations of dermatomyositis and their management. Current rheumatology reports. 2010; 12:192–7.
3. Sontheimer RD. The management of dermatomyositis: Current treatment options. Expert opinion on pharmacotherapy. 2004; 5(5):1083–99.
4. Bendewald MJ, Wetter DA, Li X, Davis MD. Incidence of dermatomyositis and clinically amyopathic dermatomyositis: A population-based study in Olmsted County, Minnesota. Archives of dermatology. 2010; 146(1):26–30.
5. Sontheimer RD. Dermatomyositis: An overview of recent progress with emphasis on dermatologic aspects. Dermatologic clinics. 2002; 20(3):387–408.
6. Bohan A, Peter JB. Polymyositis and dermatomyositis: (First of two parts). New England Journal of Medicine. 1975; 292(7):344–7.
7. Lundberg IE, Tjärnlund A, Bottai M, Werth VP, Pilkington C, Visser M de, et al. EULAR/ACR classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Annals of rheumatic diseases. 2017; 76(12):1955.
8. Ohta A, Nagai M, Nishina M, Tomimitsu H, Kohsaka H. Prevalence and incidence of polymyositis and dermatomyositis in Japan. Mod Rheumatol. 2014; 24(3):477-80.
9. Osman M, Martins KJB, Wong KO, Vu K, Guigue A, Cohen JW, et al. Incidence and prevalence, and medication use among adults living with dermatomyositis: an Alberta, Canada population-based cohort study. Sci Rep 2023; 13:16444.
10. Kronzer VL, Kimbrough BA, Crowson CS, Davis JM III, Holmqvist M, Ernste FC. Incidence, Prevalence, and Mortality of Dermatomyositis: A Population-Based Cohort Study. Arthritis Care Res 2023; 75:348-355.
11. Svensson J. Arkema EV. Lundberg IE. Holmqvist M. Incidence and prevalence of idiopathic inflammatory myopathies in Sweden: a nationwide population-based study. Rheumatology. 2017;56:802–10.
12. Tan JA, Roberts-Thomson PJ, Blumbergs P, Hakendorf P, Cox SR, Limaye V. Incidence and prevalence of idiopathic inflammatory myopathies in South Australia: a 30-year epidemiologic study of histology-proven cases. Int J Rheum Dis. 2013;16(3):331-8.
13. DeWane ME, Waldman R, Lu J. Dermatomyositis: Clinical features and pathogenesis. J Am Acad Dermatol. 2020; 82(2):267-281.
14. Abu Mansor Matardiah NH, Mohd A, Ali RA. A review of idiopathic inflammatory myopathy cases in Terengganu, Malaysia: A single centre experience. The Medical journal of Malaysia. 2012; 76:488-492.
15. Dourmishev LA. Dermatomyositis associated with malignancy. 12 case reports. Adv Exp Med Biol. 1999; 455:193-9. doi:10.1007/978-1-4615-4857-7_28.
16. Neely J, Ardalan K, Huber A, Kim S; Childhood Arthritis and Rheumatology Research Alliance Investigators. Baseline characteristics of children with juvenile dermatomyositis enrolled in the first year of the new Childhood Arthritis and Rheumatology Research Alliance registry. Pediatr Rheumatol Online J. 2022; 20(1):50. doi:10.1186/s12969-022-00709-3.
17. Nitiyarom R, Charuvanij S, Likasitwattanakul S, Thanoophunchai C, Wisuthsarewong W. Juvenile dermatomyositis in Thai children: Retrospective review of 30 cases from a tertiary care center Indian. J Dermatol Venereol Leprol 2022; 88:162-70.
18. Tong M. A Review of Dermatomyositis Cases at Hospital Besar Kuala Lumpur 1989 - 1993. Med J Malaysia. 1995; 50(1):32-26.
19. Dourmishev LA, Assen Lyubenov Dourmishev, Springerlink (Online Service. Dermatomyositis: Advances in Recognition, Understanding and Management. Berlin, Heidelberg: Springer Heidelberg; 2009. 65-68.
20. Euwer RL, Sontheimer RD. Amyopathic dermatomyositis (dermatomyositis sine myositis) Presentation of six new cases and review of the literature. J Am Acad Dermatol. 1991; 24:959–66.
21. Hoogendijk JE, Amato AA, Lecky BR, Choy EH, Lundberg IE, Rose MR, et al. 119th ENMC international workshop: trial design in adult idiopathic inflammatory myopathies, with the exception of inclusion body myositis, 10–12 October 2003, Naarden, The Netherlands. Neuromuscul Disord. 2004; 14:337–45.
22. Gerami P, Schope JM, McDonald L, Walling HW, Sontheimer RD. A systematic review of adult onset Clinically amyopathic dermatomyositis (dermatomyositis sine myositis): a missing link within the spectrum of the idiopathic inflammatory myopathies. J Am Acad Dermatol 2006; 54(4):597–613.
23. Tang K, Zhang H, Jin H. Clinical Characteristics and Management of Patients With Clinical Amyopathic Dermatomyositis: A Retrospective Study of 64 Patients at a Tertiary Dermatology Department. Front Med (Lausanne). 2021; 8:783416.
24. Rider LG, Lachenbruch PA, Monroe JB, Ravelli A, Cabalar I, Feldman BM, et al. IMACS Group. Damage extent and predictors in adult and juvenile dermatomyositis and polymyositis as determined with the myositis damage index. Arthritis Rheum. 2009; 60:3425–3435.
25. Hoesly PM, Sluzevich JC, Jambusaria-Pahlajani A, Lesser ER, Heckman MG, Abril A. Association of antinuclear antibody status with clinical features and malignancy risk in adult-onset dermatomyositis. J Am Acad Dermatol. 2019; 80(5):1364-1370.
26. Aussy A, Boyer O, Cordel N. Dermatomyositis and immune-mediated necrotizing myopathies: a window on autoimmunity and cancer. Front Immunol. 2017; 8:992
27. Chen H, Peng Q, Yang H, Yin L, Shi J, Zhang Y, et al. Increased Levels of Soluble Programmed Death Ligand 1 Associate with Malignancy in Patients with Dermatomyositis. J Rheumatol. 2018; 45(6):835-840.
28. Satoh M, Tanaka S, Ceribelli A, Calise SJ, Chan EK. A Comprehensive Overview on Myositis-Specific Antibodies: New and Old Biomarkers in Idiopathic Inflammatory Myopathy. Clin Rev Allergy Immunol. 2017; 52(1):1-19.
29. Cassius C, Le Buanec H, Bouaziz JD, Amode R. Biomarkers in Adult Dermatomyositis: Tools to Help the Diagnosis and Predict the Clinical Outcome. J Immunol Res. 2019; 2019:9141420.
30. Lu X, Yang H, Shu X, Chen F, Zhang Y, Zhang S, et al. Factors predicting malignancy in patients with polymyositis and dermatomyostis: a systematic review and meta-analysis. PLoS One. 2014; 9(4): e94128.
31. Kilinc OC, Ugurlu S. Clinical features of dermatomyositis patients with anti-TIF1 antibodies: A case based comprehensive review. Autoimmun Rev. 2023; 22(12):103464.
32. Tansley SL, Simou S, Shaddick G, Betteridge ZE, Almeida B, Gunawardena H, et al. Autoantibodies in juvenile-onset myositis: Their diagnostic value and associated clinical phenotype in a large UK cohort. J Autoimmun. 2017
33. Temmoku J, Sato S, Fujita Y, Asano T, Suzuki E, Kanno T, et al. Clinical significance of myositis-specific autoantibody profiles in Japanese patients with polymyositis/dermatomyositis. Medicine (Baltimore). 2019; 98(20):e15578.
34. Wolstencroft PW, Fiorentino DF Dermatomyositis clinical and pathological phenotypes associated with myositis-specific autoantibodies. Curr Rheumatol Rep. 2018; 20:28.
35. McCann LJ, Juggins AD, Maillard SM, Wedderburn LR, Davidson JE, Murray KJ, et al. Juvenile Dermatomyositis Research Group. The Juvenile Dermatomyositis National Registry and Repository (UK and Ireland) - clinical characteristics of children recruited within the first 5 yr. Rheumatology. 2006; 45:1255–1260.
36. Constantinides VC, Papahatzaki MM, Papadimas GK, Karandreas N, Zambelis T, Kokotis P, et al. Diagnostic Accuracy of Muscle Biopsy and Electromyography in 123 Patients with Neuromuscular Disorders. In Vivo. 2018;32(6):1647-1652.
37. Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. Lancet 2003; 362(9388):971–82
38. Kasteler JS, Callen JP. Low-dose methotrexate administered weekly is an effective corticosteroid-sparing agent for the treatment of the cutaneous manifestations of dermatomyositis. J Am Acad Dermatol. 1997; 36(1):67-71.
39. Shahin AA, Niazy MH, Haroon MM. Response to cyclophosphamide and rituximab therapy in idiopathic inflammatory myopathies: A single center experience. Egyptian Rheumatologist 2021; 43:247–51.
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