Mortality and prognostic factors in idiopathic inflammatory myositis: a retrospective analysis of a large multicenter cohort of Spain

• Published in: Rheumatology international Vol. 37; no. 11; pp. 1853 - 1861
• Main Authors: Nuño-Nuño, Laura, Joven, Beatriz Esther, Carreira, Patricia E., Maldonado-Romero, Valentina, Larena-Grijalba, Carmen, Cubas, Irene Llorente, Tomero, Eva Gloria, Barbadillo-Mateos, María Carmen, De la Peña Lefebvre, Paloma García, Ruiz-Gutiérrez, Lucía, López-Robledillo, Juan Carlos, Moruno-Cruz, Henry, Pérez, Ana, Cobo-Ibáñez, Tatiana, Almodóvar González, Raquel, Lojo, Leticia, García De Yébenes, María Jesús, López-Longo, Francisco Javier
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01.11.2017; Springer Nature B.V
• Subjects: Adult; Aged; Cause of Death; Female; Humans; Inflammatory diseases; Male; Medical prognosis; Medicine; Medicine & Public Health; Middle Aged; Mortality; Musculoskeletal diseases; Myositis - mortality; Observational Research; Prognosis; Retrospective Studies; Rheumatology; Spain; Idiopathic inflammatory myositis; Prognosis; Overlap myositis; Survival
• ISSN: 0172-8172; 1437-160X; 1437-160X
• DOI: 10.1007/s00296-017-3799-x

The present study was undertaken to assess mortality, causes of death, and associated prognostic factors in a large cohort of patients diagnosed with idiopathic inflammatory myositis (IIM) from Spain. A retrospective longitudinal study was carried out in 467 consecutive patients with IIM, identified from 12 medical centers. Patients were classified as primary polymyositis, primary dermatomyositis (DM), overlap myositis, cancer-associated myositis (CAM), and juvenile idiopathic inflammatory myopathies. A total of 113 deaths occurred (24%) after a median follow-up time of 9.7 years. In the overall cohort, the 2-, 5-, and 10-year survival probabilities were 91.9, 86.7, and 77%, respectively. Main causes of death were infections and cancer (24% each). Multivariate model revealed that CAM (HR = 24.06), OM (HR = 12.00), DM (HR = 7.26), higher age at diagnosis (HR = 1.02), severe infections (HR = 3.66), interstitial lung disease (HR = 1.61), and baseline elevation of acute phase reactants (HR = 3.03) were associated with a worse prognosis, while edema of the hands (HR = 0.39), female gender (HR = 0.39), and longer disease duration (HR = 0.73) were associated with a better prognosis. The standardized mortality ratio was 1.56 (95% CI 1.28–1.87) compared to the Spanish general population. Our findings indicate that IIM has a high long-term mortality, with an excess of mortality compared to the Spanish population. A more aggressive therapy may be required in IIM patients presenting with poor predictive factors.