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Clinical spectrums and outcomes of necrotizing autoimmune myopathy versus other idiopathic inflammatory myopathies: a multicenter case-control study

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Abstract

Objective

To investigate the clinical characteristics, laboratory features, and treatment outcomes of Thai patients compared between those with necrotizing autoimmune myopathy (NAM) and those with other idiopathic inflammatory myopathies (IIMs) or non-NAM.

Methods

This multicenter case-control study included patients aged ≥ 18 years who were diagnosed with IIMs by muscle pathology, and who had relevant clinical and laboratory data, including muscle enzymes, from at least 3 follow-up visits during a 1-year period. Baseline clinical and laboratory data were recorded. Serum myositis-specific autoantibodies (MSAs) were obtained on the date of recruitment.

Results

Of the 70 included patients, 67% had NAM, and 33% had non-NAM. The mean age of patients was 50.5 ± 15.9 years, 67% were female, and the median duration of symptoms was 2 months (IQR, 1–4). History of cancer was significantly higher in non-NAM (21.7% vs. 2.1%, p = 0.01). Gottron’s papules were significantly more prevalent in non-NAM (21.7% vs. 4.3%, p = 0.04). Non-NAM had a higher prevalence of anti-Mi-2a (17.4% vs. 2.1%, p = 0.04) and Mi-2b (17.4% vs. 0.0%, p = 0.01); however, the presence of other MSAs, including anti-HMGCR and anti-SRP, was similar between groups. Improvement in motor power and treatment intensification with glucocorticoid and/or immunosuppressive agents 3 times throughout the follow-up period was similar between groups (NAM 46.8% vs. non-NAM 34.8%, p = 0.34).

Conclusion

NAM is indistinguishable from non-NAM by clinical manifestations, serology, or laboratory findings, except that pathognomonic skin sign of Gottron’s papules and anti-Mi2 are suggestive of dermatomyositis. The integration of clinical, serological, and pathological data is essential for making a diagnosis of NAM.

Key Points

• NAM is indistinguishable from non-NAM by clinical manifestations, serology, or laboratory findings.

• The integration of clinical, serological, and pathological data is essential for making a diagnosis of NAM.

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Acknowledgments

The authors gratefully acknowledge Mrs. Phakhamon Thaweeratthakul, BSc (nursing), MSc (public health), for blood sample collection; Ms. Khemajira Karaketklang, BSc (nursing), MPH, for statistical analysis; Assoc. Prof. Ronnachai Viriyataweekul, MD, of the Department of Clinical Pathology for technical support; and Mr. Chakriwong Ma-on and Ms. Ampa Changnam of the Department of Pathology for slide preparation.

Funding

This study was funded by a Siriraj Research Grant from the Faculty of Medicine Siriraj Hospital, Mahidol University (grant no. R 016132020).

Author information

Authors and Affiliations

  1. Division of Rheumatology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, 2 Wanglang Road, Bangkoknoi, Bangkok, 10700, Thailand

    Khemmapop Yongchairat & Wanruchada Katchamart

  2. Department of Pathology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand

    Jantima Tanboon

  3. Department of Pathology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand

    Jariya Waisayarat

  4. Rheumatic Disease Unit, Department of Internal Medicine, Phramongkutklao Hospital and Phramongkutklao College of Medicine, Bangkok, Thailand

    Pongthorn Narongroeknawin

  5. Division of Allergy, Immunology, and Rheumatology, Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand

    Parawee Chevaisrakul

  6. Division of Neurology, Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand

    Charungthai Dejthevaporn

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  1. Khemmapop Yongchairat
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Contributions

All authors made substantial contributions to the conception, design of the work, the acquisition, analysis, interpretation of data, drafted the work or substantively revised it, and approved the submitted version.

Corresponding author

Correspondence to Wanruchada Katchamart.

Ethics declarations

The protocol for this study was approved by the Institutional Review Board (SIRB) of the Faculty of Medicine Siriraj Hospital, Mahidol University (COA no. SI 040/2018), the Ethical Clearance Committee on Human Rights Related to Research Involving Human Subjects of the Faculty of Medicine Ramathibodi Hospital, Mahidol University (MURA) (COA no. 2018/57), and the Institutional Review Board Committee of the Royal Thai Army Medical Department (IRBRTA) (COA no. S068h/60). All procedures performed in this study that involved human participants were in accordance with the ethical standards of the institutional and/or national research committee, and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standard. Written informed consent was obtained from all individual participants included in this study.

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Yongchairat, K., Tanboon, J., Waisayarat, J. et al. Clinical spectrums and outcomes of necrotizing autoimmune myopathy versus other idiopathic inflammatory myopathies: a multicenter case-control study. Clin Rheumatol 38, 3459–3469 (2019). https://doi.org/10.1007/s10067-019-04756-2

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