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

Authors
  • Yongchairat, Khemmapop1
  • Tanboon, Jantima2
  • Waisayarat, Jariya3
  • Narongroeknawin, Pongthorn4
  • Chevaisrakul, Parawee5
  • Dejthevaporn, Charungthai6
  • Katchamart, Wanruchada7
  • 1 Division of Rheumatology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, 2 Wanglang Road, Bangkoknoi, Bangkok, 10700, Thailand. , (Thailand)
  • 2 Department of Pathology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand. , (Thailand)
  • 3 Department of Pathology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand. , (Thailand)
  • 4 Rheumatic Disease Unit, Department of Internal Medicine, Phramongkutklao Hospital and Phramongkutklao College of Medicine, Bangkok, Thailand. , (Thailand)
  • 5 Division of Allergy, Immunology, and Rheumatology, Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand. , (Thailand)
  • 6 Division of Neurology, Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand. , (Thailand)
  • 7 Division of Rheumatology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, 2 Wanglang Road, Bangkoknoi, Bangkok, 10700, Thailand. [email protected] , (Chad)
Type
Published Article
Journal
Clinical Rheumatology
Publisher
Springer-Verlag
Publication Date
Dec 01, 2019
Volume
38
Issue
12
Pages
3459–3469
Identifiers
DOI: 10.1007/s10067-019-04756-2
PMID: 31446540
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

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. 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. 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). 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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