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Year 2025, Volume: 16 Issue: 55, 109 - 115, 18.08.2025
https://doi.org/10.17944/interdiscip.1699543

Abstract

References

  • Lundberg IE, Tjärnlund A, Bottai M, Werth VP, Pilkington C, de Visser 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. Arthritis Rheumatol. 2017;69(12):2271-82. https://doi.org/10.1002/art.40320
  • Cavazzana I, Fredi M, Selmi C, Tincani A, Franceschini F. The Clinical and Histological Spectrum of Idiopathic Inflammatory Myopathies. Clin Rev Allergy Immunol. 2017;52(1):88-98. https://doi.org/10.1007/s12016-015-8517-4
  • Albrecht I, Wick C, Hallgren Å, Tjärnlund A, Nagaraju K, Andrade F, et al. Development of autoantibodies against muscle-specific FHL1 in severe inflammatory myopathies. J Clin Invest. 2015;125(12):4612-24. https://doi.org/10.1172/JCI81031
  • Paramalingam S, Needham M, Raymond W, Mastaglia F, Lightowler D, Morin N, et al. Muscle shear wave elastography, conventional B mode and power doppler ultrasonography in healthy adults and patients with autoimmune inflammatory myopathies: a pilot cross-sectional study. BMC Musculoskelet Disord. 2021;22(1):537. https://doi.org/10.1186/s12891-021-04424-0
  • Bhardwaj M, Khamankar SG, Sinwal A, Poonia R, Sinwal V. A Review of Recent Advances in the Classification, Pathogenesis, Diagnosis, and Treatment of Idiopathic Inflammatory Myopathies. Journal of Pharma Insights and Research. 2024;2(6):205-14. https://doi.org/10.69613/yd1kff94
  • Allameen NA, Ramos-Lisbona AI, Wedderburn LR, Lundberg IE, Isenberg DA. An update on autoantibodies in the idiopathic inflammatory myopathies. Nat Rev Rheumatol. 2025;21(1):46-62. https://doi.org/10.1038/s41584-024-01188-4
  • Patel P, Marinock JM, Ajmeri A, Brent LH. A Review of Antisynthetase Syndrome-Associated Interstitial Lung Disease. Int J Mol Sci. 2024;25(8). https://doi.org/10.3390/ijms25084453
  • Uruha A, Suzuki S, Nishino I. Diagnosis of dermatomyositis: autoantibody profile and muscle pathology. Clinical and Experimental Neuroimmunology. 2017;8(4):302-12. https://doi.org/10.1111/cen3.12419
  • Ferreira BSA, Cunha BMD, Moreira LA, Fonseca M, Cavalcante EBU. Comments: Myositis-specific and myositis-associated autoantibody profiles and their clinical associations in a large series of patients with polymyositis and dermatomyositis. Clinics (Sao Paulo). 2022;77:100077. https://doi.org/10.1016/j.clinsp.2022.100077
  • Alonso-Larruga A, Bustabad S, Navarro-Gonzálvez JA, Rodríguez-Lozano B, Franco A, Barrios Y. Isolated Ro52 Antibodies as Immunological Marker of a Mild Phenotype of Undifferentiated Connective Tissue Diseases. Int J Rheumatol. 2017;2017:3076017. https://doi.org/10.1155/2017/3076017
  • Yang H, Lu X, Peng Q, Jiang W, Shi J, Zhang Y, et al. Differential Clinical Associations of Anti-Nuclear Matrix Protein 2 Autoantibodies in Patients With Idiopathic Inflammatory Myopathies. Arthritis Rheumatol. 2018;70(8):1288-97. https://doi.org/10.1002/art.40491
  • Pinal-Fernandez I, Muñoz-Braceras S, Casal-Dominguez M, Pak K, Torres-Ruiz J, Musai J, et al. Pathological autoantibody internalisation in myositis. Annals of the rheumatic diseases. 2024;83(11):1549-60. https://doi.org/10.1136/ard-2024-225773
  • Council MR. Aids to the examination of the peripheral nervous system. London: Her Majesty’s Stationary Office. 1976.
  • Dalakas MC. Inflammatory muscle diseases. N Engl J Med. 2015;372(18):1734-47. https://doi.org/10.1056/NEJMra1402225
  • Rider LG, Miller FW. Deciphering the clinical presentations, pathogenesis, and treatment of the idiopathic inflammatory myopathies. Jama. 2011;305(2):183-90. https://doi.org/10.1001/jama.2010.1977
  • Dimachkie MM, Barohn RJ. Idiopathic inflammatory myopathies. Semin Neurol. 2012;32(3):227-36. https://doi.org/10.1055/s-0032-1329201
  • Greenberg SA. Inflammatory myopathies: evaluation and management. Semin Neurol. 2008;28(2):241-9. https://doi.org/10.1055/s-2008-1062267
  • Allameen NA, Ramos-Lisbona AI, Wedderburn LR, Lundberg IE, Isenberg DA. An update on autoantibodies in the idiopathic inflammatory myopathies. Nature Reviews Rheumatology. 2025;21(1):46-62. https://doi.org/10.1038/s41584-024-01188-4
  • Shu XM, Lu X, Xie Y, Wang GC. Clinical characteristics and favorable long-term outcomes for patients with idiopathic inflammatory myopathies: a retrospective single center study in China. BMC Neurology. 2011;11:1-11. https://doi.org/10.1186/1471-2377-11-143
  • Deroo L, Achten H, De Boeck K, Genbrugge E, Bauters W, Roels D, et al. The value of separate detection of anti-Ro52, anti-Ro60 and anti-SSB/La reactivities in relation to diagnosis and phenotypes in primary Sjögren’s syndrome. Clin Exp Rheumatol. 2022;40(12):2310-7. https://doi.org/10.55563/clinexprheumatol/l70874
  • Sun KY, Fan Y, Wang YX, Zhong YJ, Wang GF. Prevalence of interstitial lung disease in polymyositis and dermatomyositis: A meta-analysis from 2000 to 2020. Semin Arthritis Rheum. 2021;51(1):175-91. https://doi.org/10.1016/j.semarthrit.2020.11.009
  • 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(5):337-45. https://doi.org/10.1016/j.nmd.2004.02.006
  • Mammen AL. Autoimmune myopathies: autoantibodies, phenotypes and pathogenesis. Nat Rev Neurol. 2011;7(6):343-54. https://doi.org/10.1038/nrneurol.2011.63
  • Pipitone N, Salvarani C. Up-to-date treatment and management of myositis. Curr Opin Rheumatol. 2020;32(6):523-7.https://doi.org/10.1097/BOR.0000000000000745
  • Zhen C, Hou Y, Zhao B, Ma X, Dai T, Yan C. Efficacy and safety of rituximab treatment in patients with idiopathic inflammatory myopathies: A systematic review and meta-analysis. Front Immunol. 2022;13:1051609. https://doi.org/10.3389/fimmu.2022.1051609
  • Tsamis KI, Boutsoras C, Kaltsonoudis E, Pelechas E, Nikas IP, Simos YV, et al. Clinical features and diagnostic tools in idiopathic inflammatory myopathies. Crit Rev Clin Lab Sci. 2022;59(4):219-40. https://doi.org/10.1080/10408363.2021.2000584
  • Connolly CM, Gupta L, Fujimoto M, Machado PM, Paik JJ. Idiopathic inflammatory myopathies: current insights and future frontiers. The Lancet Rheumatology. 2024;6(2):e115-e27. https://doi.org/10.1016/S2665-9913(23)00322-3
  • Saygin D, Glaubitz S, Zeng R, Bottai M, de Visser M, Dimachkie MM, et al. Performance of the 2017 EULAR/ACR Classification Criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups: a scoping review. Clin Exp Rheumatol. 2024;42(2):403-12. https://doi.org/10.55563/clinexprheumatol/vuc5py
  • Mohan N, Yoganandan D, Mohanasundaram K, Rajalakshmi KV, Kumar SM. Rapidly progressing treatment resistant anti-TIF1 gamma antibody positive dermatomyositis. International Journal of Advances in Medicine. 2022;9(3):1. https://dx.doi.org/10.18203/2349-3933.ijam20220358
  • Vincze A, Gaál J, Griger Z. Bone Health in Idiopathic Inflammatory Myopathies: Diagnosis and Management. Curr Rheumatol Rep. 2021;23(7):55. https://doi.org/10.1007/s11926-021-01016-8

The clinical features and therapeutic results in patients diagnosed with idiopathic inflammatory myositis: single center experience

Year 2025, Volume: 16 Issue: 55, 109 - 115, 18.08.2025
https://doi.org/10.17944/interdiscip.1699543

Abstract

Objective: This study assessed clinical features, laboratory profiles, and treatment outcomes in idiopathic inflammatory myopathies (IIMs) patients after six months of immunosuppression.
Method: Retrospective analysis occurred at a tertiary rheumatology center. Baseline and six-month post-therapy assessments were conducted on patients diagnosed with polymyositis or dermatomyositis. Records were kept of clinical symptoms, laboratory markers, and muscle strength scores.
Results: With a mean age of 43±11.1 years and an almost even gender distribution (48.4% female), 31 patients were enrolled. Of these individuals, 87.1% were diagnosed with polymyositis and 12.9% with dermatomyositis. At baseline, the predominant symptom was muscle weakness (77.4%), alongside notably high levels of muscle enzymes (median creatinine kinase [CK]: 3340 IU/L) and inflammatory markers (C-reactive protein [CRP]: 11.4 mg/L, erythrocyte sedimentation rate [ESR]: 39 mm/h). Positivity for Jo-1 and Ro52 autoantibodies was found in 16.1% of the cases. Following six months of treatment, there were significant reductions in all laboratory markers (p<0.001), and all patients exhibited improved muscle strength (5/5).
Conclusion: Patients with IIMs experience significant biochemical and clinical improvement with immunosuppressive therapy. Optimal recovery and long-term muscle function depend on early diagnosis and personalized treatments.

References

  • Lundberg IE, Tjärnlund A, Bottai M, Werth VP, Pilkington C, de Visser 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. Arthritis Rheumatol. 2017;69(12):2271-82. https://doi.org/10.1002/art.40320
  • Cavazzana I, Fredi M, Selmi C, Tincani A, Franceschini F. The Clinical and Histological Spectrum of Idiopathic Inflammatory Myopathies. Clin Rev Allergy Immunol. 2017;52(1):88-98. https://doi.org/10.1007/s12016-015-8517-4
  • Albrecht I, Wick C, Hallgren Å, Tjärnlund A, Nagaraju K, Andrade F, et al. Development of autoantibodies against muscle-specific FHL1 in severe inflammatory myopathies. J Clin Invest. 2015;125(12):4612-24. https://doi.org/10.1172/JCI81031
  • Paramalingam S, Needham M, Raymond W, Mastaglia F, Lightowler D, Morin N, et al. Muscle shear wave elastography, conventional B mode and power doppler ultrasonography in healthy adults and patients with autoimmune inflammatory myopathies: a pilot cross-sectional study. BMC Musculoskelet Disord. 2021;22(1):537. https://doi.org/10.1186/s12891-021-04424-0
  • Bhardwaj M, Khamankar SG, Sinwal A, Poonia R, Sinwal V. A Review of Recent Advances in the Classification, Pathogenesis, Diagnosis, and Treatment of Idiopathic Inflammatory Myopathies. Journal of Pharma Insights and Research. 2024;2(6):205-14. https://doi.org/10.69613/yd1kff94
  • Allameen NA, Ramos-Lisbona AI, Wedderburn LR, Lundberg IE, Isenberg DA. An update on autoantibodies in the idiopathic inflammatory myopathies. Nat Rev Rheumatol. 2025;21(1):46-62. https://doi.org/10.1038/s41584-024-01188-4
  • Patel P, Marinock JM, Ajmeri A, Brent LH. A Review of Antisynthetase Syndrome-Associated Interstitial Lung Disease. Int J Mol Sci. 2024;25(8). https://doi.org/10.3390/ijms25084453
  • Uruha A, Suzuki S, Nishino I. Diagnosis of dermatomyositis: autoantibody profile and muscle pathology. Clinical and Experimental Neuroimmunology. 2017;8(4):302-12. https://doi.org/10.1111/cen3.12419
  • Ferreira BSA, Cunha BMD, Moreira LA, Fonseca M, Cavalcante EBU. Comments: Myositis-specific and myositis-associated autoantibody profiles and their clinical associations in a large series of patients with polymyositis and dermatomyositis. Clinics (Sao Paulo). 2022;77:100077. https://doi.org/10.1016/j.clinsp.2022.100077
  • Alonso-Larruga A, Bustabad S, Navarro-Gonzálvez JA, Rodríguez-Lozano B, Franco A, Barrios Y. Isolated Ro52 Antibodies as Immunological Marker of a Mild Phenotype of Undifferentiated Connective Tissue Diseases. Int J Rheumatol. 2017;2017:3076017. https://doi.org/10.1155/2017/3076017
  • Yang H, Lu X, Peng Q, Jiang W, Shi J, Zhang Y, et al. Differential Clinical Associations of Anti-Nuclear Matrix Protein 2 Autoantibodies in Patients With Idiopathic Inflammatory Myopathies. Arthritis Rheumatol. 2018;70(8):1288-97. https://doi.org/10.1002/art.40491
  • Pinal-Fernandez I, Muñoz-Braceras S, Casal-Dominguez M, Pak K, Torres-Ruiz J, Musai J, et al. Pathological autoantibody internalisation in myositis. Annals of the rheumatic diseases. 2024;83(11):1549-60. https://doi.org/10.1136/ard-2024-225773
  • Council MR. Aids to the examination of the peripheral nervous system. London: Her Majesty’s Stationary Office. 1976.
  • Dalakas MC. Inflammatory muscle diseases. N Engl J Med. 2015;372(18):1734-47. https://doi.org/10.1056/NEJMra1402225
  • Rider LG, Miller FW. Deciphering the clinical presentations, pathogenesis, and treatment of the idiopathic inflammatory myopathies. Jama. 2011;305(2):183-90. https://doi.org/10.1001/jama.2010.1977
  • Dimachkie MM, Barohn RJ. Idiopathic inflammatory myopathies. Semin Neurol. 2012;32(3):227-36. https://doi.org/10.1055/s-0032-1329201
  • Greenberg SA. Inflammatory myopathies: evaluation and management. Semin Neurol. 2008;28(2):241-9. https://doi.org/10.1055/s-2008-1062267
  • Allameen NA, Ramos-Lisbona AI, Wedderburn LR, Lundberg IE, Isenberg DA. An update on autoantibodies in the idiopathic inflammatory myopathies. Nature Reviews Rheumatology. 2025;21(1):46-62. https://doi.org/10.1038/s41584-024-01188-4
  • Shu XM, Lu X, Xie Y, Wang GC. Clinical characteristics and favorable long-term outcomes for patients with idiopathic inflammatory myopathies: a retrospective single center study in China. BMC Neurology. 2011;11:1-11. https://doi.org/10.1186/1471-2377-11-143
  • Deroo L, Achten H, De Boeck K, Genbrugge E, Bauters W, Roels D, et al. The value of separate detection of anti-Ro52, anti-Ro60 and anti-SSB/La reactivities in relation to diagnosis and phenotypes in primary Sjögren’s syndrome. Clin Exp Rheumatol. 2022;40(12):2310-7. https://doi.org/10.55563/clinexprheumatol/l70874
  • Sun KY, Fan Y, Wang YX, Zhong YJ, Wang GF. Prevalence of interstitial lung disease in polymyositis and dermatomyositis: A meta-analysis from 2000 to 2020. Semin Arthritis Rheum. 2021;51(1):175-91. https://doi.org/10.1016/j.semarthrit.2020.11.009
  • 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(5):337-45. https://doi.org/10.1016/j.nmd.2004.02.006
  • Mammen AL. Autoimmune myopathies: autoantibodies, phenotypes and pathogenesis. Nat Rev Neurol. 2011;7(6):343-54. https://doi.org/10.1038/nrneurol.2011.63
  • Pipitone N, Salvarani C. Up-to-date treatment and management of myositis. Curr Opin Rheumatol. 2020;32(6):523-7.https://doi.org/10.1097/BOR.0000000000000745
  • Zhen C, Hou Y, Zhao B, Ma X, Dai T, Yan C. Efficacy and safety of rituximab treatment in patients with idiopathic inflammatory myopathies: A systematic review and meta-analysis. Front Immunol. 2022;13:1051609. https://doi.org/10.3389/fimmu.2022.1051609
  • Tsamis KI, Boutsoras C, Kaltsonoudis E, Pelechas E, Nikas IP, Simos YV, et al. Clinical features and diagnostic tools in idiopathic inflammatory myopathies. Crit Rev Clin Lab Sci. 2022;59(4):219-40. https://doi.org/10.1080/10408363.2021.2000584
  • Connolly CM, Gupta L, Fujimoto M, Machado PM, Paik JJ. Idiopathic inflammatory myopathies: current insights and future frontiers. The Lancet Rheumatology. 2024;6(2):e115-e27. https://doi.org/10.1016/S2665-9913(23)00322-3
  • Saygin D, Glaubitz S, Zeng R, Bottai M, de Visser M, Dimachkie MM, et al. Performance of the 2017 EULAR/ACR Classification Criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups: a scoping review. Clin Exp Rheumatol. 2024;42(2):403-12. https://doi.org/10.55563/clinexprheumatol/vuc5py
  • Mohan N, Yoganandan D, Mohanasundaram K, Rajalakshmi KV, Kumar SM. Rapidly progressing treatment resistant anti-TIF1 gamma antibody positive dermatomyositis. International Journal of Advances in Medicine. 2022;9(3):1. https://dx.doi.org/10.18203/2349-3933.ijam20220358
  • Vincze A, Gaál J, Griger Z. Bone Health in Idiopathic Inflammatory Myopathies: Diagnosis and Management. Curr Rheumatol Rep. 2021;23(7):55. https://doi.org/10.1007/s11926-021-01016-8
There are 30 citations in total.

Details

Primary Language English
Subjects ​Internal Diseases
Journal Section Research Articles
Authors

Fatih Albayrak

Arif Babayiğit

Neşe Çabuk Çelik

Ali Şahin

Publication Date August 18, 2025
Submission Date May 14, 2025
Acceptance Date August 4, 2025
Published in Issue Year 2025 Volume: 16 Issue: 55

Cite

Vancouver Albayrak F, Babayiğit A, Çabuk Çelik N, Şahin A. The clinical features and therapeutic results in patients diagnosed with idiopathic inflammatory myositis: single center experience. Interdiscip Med J. 2025;16(55):109-15.