Inflammatory myopathies

Long‐Term Outcomes and Prognosis Factors in Patients With Idiopathic Inflammatory Myopathies Based on Myositis‐Specific Autoantibodies: A Single Cohort Study

Arthritis Care Res (Hoboken)Published Cohort study

Authors' conclusion

Despite favorable overall survival in patients with IIM, the anti-MDA-5-positive subgroup had the highest mortality rate among all MSA subgroups, highlighting the distinctive prognosis for patients with different MSAs. RP-ILD and malignancy are the most common causes of death in IIM patients.

Study context

Population
Patients with IIM stratified by myositis-specific antibody (MSA)
Participants
628 patients
Exposure
Survival and prognostic factors by MSA subgroup
Outcome
Long-term survival
Follow-up
1, 5 and 10 years
Methodological detailssingle cohort, Kaplan-Meier survival analysis, univariate and multivariate Cox regression
  • Single cohort
  • Kaplan-Meier survival analysis
  • Univariate and multivariate Cox regression

Main finding

As reported in the abstract

Cumulative survival, IIM overall
1-year survival, overall
91.4%
5-year survival, overall
82.8%
10-year survival, overall
75.6%
Survival differed significantly across MSA subsets
P < 0.001
1-year survival, anti-MDA-5-positive (lowest of all subgroups)
79.5%
10-year survival, anti-MDA-5-positive (lowest of all subgroups)
58.5%
10-year survival, anti-signal recognition particle (anti-SRP)-positive (highest)
96.4%

Observational data: associations, not effects.

Further findings

Independent prognostic factors for long-term prognosis, IIM overall (multivariate Cox)

Rapidly progressive interstitial lung disease (RP-ILD), HR (P < 0.01)
17.47
Malignancy, HR (P < 0.01)
20.36
Elevated serum ferritin, HR (P < 0.01)
9.15
Disease duration (protective), HR (P = 0.003)
0.27

Independent risk factors for death by MSA subgroup

RP-ILD in anti-MDA-5-positive, HR (P = 0.017)
3.4
Malignancy in anti-aminoacyl-tRNA synthetase antibody-positive, HR (P < 0.001)
46.69
Malignancy in anti-TIF1γ-positive, HR (P < 0.001)
6.65
Malignancy in MSA-negative, HR (P < 0.001)
4.48
RP-ILD in MSA-negative, HR (P < 0.001)
72.28

Original publication

Long‐Term Outcomes and Prognosis Factors in Patients With Idiopathic Inflammatory Myopathies Based on Myositis‐Specific Autoantibodies: A Single Cohort Study. Arthritis Care Res (Hoboken). Published 11 December 2022. doi:10.1002/acr.24993

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