Differences in the autoantibody phenotypes and long-term outcomes between juvenile- and adult-idiopathic inflammatory myopathies
Authors' conclusion
Drug-free condition was achieved more frequently in juvenile-IIM patients than adult-IIM patients. Specific autoantibodies were associated with different clinical characteristics and outcomes between juvenile-IIM and adult-IIM.
Study context
- Population
- Japanese IIM patients, Kyoto University Registry
- Participants
- 320 patients
- Exposure
- Juvenile-IIM vs adult-IIM
- Juvenile-IIMn = 34
- Adult-IIMn = 286reference
- Outcome
- Cumulative drug-free condition rate
- Follow-up
- Up to 20 years
Methodological detailsregistry-based (Kyoto University Registry), follow-up up to 20 years
- Registry-based (Kyoto University Registry)
- Follow-up up to 20 years
Main finding
As reported in the abstract
P = 0.0016
Observational data: associations, not effects.
Further findings
Autoantibody frequencies
anti-TIF1-γ
anti-MDA-5
anti-ARS
anti-NXP-2
anti-SRP Adult-IIM 8 %
anti-Mi-2 Adult-IIM 2 %
Anti-TIF1-γ subgroup: muscle symptoms, malignancy, GC use, drug-free condition
Muscle symptoms
Malignancy
GC use
Drug-free condition
Anti-MDA-5 subgroup: drug-free condition
Anti-ARS subgroup: drug-free condition
Anti-NXP-2 subgroup: drug-free condition
Anti-MDA-5 subgroup: drug-free condition
- Both groups: high frequency of amyopathic DM, interstitial lung disease (ILD) and multi-immunosuppressive therapy
Anti-ARS subgroup: drug-free condition
- Both groups: frequent skin rashes, muscle symptoms, ILD and need for multi-immunosuppressive therapy
Anti-NXP-2 subgroup: drug-free condition
- Both groups: frequent skin rashes and muscle symptoms, low ILD frequency, frequent MTX and GC use
Original publication
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