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

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Abstract

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.

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References

  1. Meyer A, Meyer N, Schaeffer M, Gottenberg JE, Geny B, Sibilia J (2015) Incidence and prevalence of inflammatory myopathies: a systematic review. Rheumatology (Oxford) 54:50–63

    Article  CAS  Google Scholar 

  2. Mastaglia FL, Phillips BA (2002) Idiopathic inflammatory myopathies: epidemiology, classification, and diagnostic criteria. Rheum Dis Clin North Am 28:723–741

    Article  PubMed  Google Scholar 

  3. Medsger TA Jr, Robinson H, Masi AT (1971) Factors affecting survivorship in polymyositis. A life-table study of 124 patients. Arthritis Rheum 14:249–258

    Article  PubMed  Google Scholar 

  4. Benbassat J, Gefel D, Larholt K, Sukenik S, Morgenstern V, Zlotnick A (1985) Prognostic factors in polymyositis/dermatomyositis. A computer-assisted analysis of ninety-two cases. Arthritis Rheum 28:249–255

    Article  CAS  PubMed  Google Scholar 

  5. Danko K, Ponyi A, Constantin T, Borgulya G, Szegedi G (2004) Long-term survival of patients with idiopathic inflammatory myopathies according to clinical features: a longitudinal study of 162 cases. Medicine (Baltimore) 83:35–42

    Article  Google Scholar 

  6. Lundberg IE, Forbess CJ (2008) Mortality in idiopathic inflammatory myopathies. Clin Exp Rheumatol 26:S109–S114

    CAS  PubMed  Google Scholar 

  7. Torres C, Belmonte R, Carmona L et al (2006) Survival, mortality and causes of death in inflammatory myopathies. Autoimmunity 39:205–215

    Article  PubMed  Google Scholar 

  8. Cavagna L, Nuno L, Scire CA et al (2015) Clinical spectrum time course in anti Jo-1 positive antisynthetase syndrome: results from an international retrospective multicenter study. Medicine (Baltimore) 94:e1144

    Article  CAS  Google Scholar 

  9. Airio A, Kautiainen H, Hakala M (2006) Prognosis and mortality of polymyositis and dermatomyositis patients. Clin Rheumatol 25:234–239

    Article  CAS  PubMed  Google Scholar 

  10. Bhansing KJ, van Riel PL, van Engelen BG, Fransen J, Vonk MC (2016) Patients with systemic sclerosis/polymyositis overlap have a worse survival rate than patients without it. J Rheumatol 43:1838–1843

    Article  PubMed  Google Scholar 

  11. Nuño L, Joven B, Carreira P (2016) Multicenter registry on inflammatory myositis from the rheumatology society in Madrid, Spain: descriptive analysis. Reumatol Clin. doi:10.1016/j.reuma.2016.07.010

    PubMed  Google Scholar 

  12. Bohan A, Peter JB (1975) Polymyositis and dermatomyositis (first of two parts). N Engl J Med 292:344–347

    Article  CAS  PubMed  Google Scholar 

  13. Tanimoto K, Nakano K, Kano S et al (1995) Classification criteria for polymyositis and dermatomyositis. J Rheumatol 22:668–674

    CAS  PubMed  Google Scholar 

  14. Arnett FC, Edworthy SM, The Bloch DA, Association American Rheumatism et al (1987) revised criteria for the classification of rheumatoid arthritis. Arthritis Rheum 31:315–324

    Article  Google Scholar 

  15. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American rheumatism association diagnostic and therapeutic criteria committee. Arthritis Rheum. 23:581–590

  16. Hochberg MC (1997) Updating the American college of rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 40:1725

    Article  CAS  PubMed  Google Scholar 

  17. Alarcon-Segovia D, Cardiel MH (1989) Comparison between 3 diagnostic criteria for mixed connective tissue disease. Study of 593 patients. J Rheumatol 16:328–334

    CAS  PubMed  Google Scholar 

  18. Fox RI, Robinson CA, Curd JG, Kozin F, Howell FV (1986) Sjogren’s syndrome. Proposed criteria for classification. Arthritis Rheum 29:577–585

    Article  CAS  PubMed  Google Scholar 

  19. Andras C, Ponyi A, Constantin T et al (2008) Dermatomyositis and polymyositis associated with malignancy: a 21-year retrospective study. J Rheumatol 35:438–444

    PubMed  Google Scholar 

  20. Danieli MG, Gambini S, Pettinari L, Logullo F, Veronesi G, Gabrielli A (2014) A impact of treatment on survival in polymyositis and dermatomyositis. A single-centre long-term follow-up study. Autoimmun Rev 13:1048–1054

    Article  PubMed  Google Scholar 

  21. Marie I (2012) Morbidity and mortality in adult polymyositis and dermatomyositis. Curr Rheumatol Rep 14:275–285

    Article  CAS  PubMed  Google Scholar 

  22. Yamasaki Y, Yamada H, Ohkubo M et al (2011) Longterm survival and associated risk factors in patients with adult-onset idiopathic inflammatory myopathies and amyopathic dermatomyositis: experience in a single institute in Japan. J Rheumatol 38:1636–1643

    Article  CAS  PubMed  Google Scholar 

  23. Tiniakou E, Mammen AL (2017) Idiopathic inflammatory myopathies and malignancy: a comprehensive review. Clin Rev Allergy Immunol 52:20–33

    Article  CAS  PubMed  Google Scholar 

  24. Amaral Silva M, Cogollo E, Isenberg DA (2016) Why do patients with myositis die? A retrospective analysis of a single-centre cohort. Clin Exp Rheumatol 34:820–826

    PubMed  Google Scholar 

  25. Marie I, Hachulla E, Hatron PY et al (2001) Polymyositis and dermatomyositis: short term and longterm outcome, and predictive factors of prognosis. J Rheumatol 28:2230–2237

    CAS  PubMed  Google Scholar 

  26. Sultan SM, Ioannou Y, Moss K, Isenberg DA (2002) Outcome in patients with idiopathic inflammatory myositis: morbidity and mortality. Rheumatology (Oxford) 41:22–26

    Article  CAS  Google Scholar 

  27. Limaye V, Hakendorf P, Woodman RJ, Blumbergs P, Roberts-Thomson P (2012) Mortality and its predominant causes in a large cohort of patients with biopsy-determined inflammatory myositis. Intern Med J 42:191–198

    Article  CAS  PubMed  Google Scholar 

  28. Marie I, Menard JF, Hachulla E et al (2011) Infectious complications in polymyositis and dermatomyositis: a series of 279 patients. Semin Arthritis Rheum 41:48–60

    Article  PubMed  Google Scholar 

  29. Ishizuka M, Watanabe R, Ishii T et al (2016) Long-term follow-up of 124 patients with polymyositis and dermatomyositis: statistical analysis of prognostic factors. Mod Rheumatol 26:115–120

    Article  PubMed  Google Scholar 

  30. Marie I, Hatron PY, Dominique S, Cherin P, Mouthon L, Menard JF (2011) Short-term and long-term outcomes of interstitial lung disease in polymyositis and dermatomyositis: a series of 107 patients. Arthritis Rheum 63:3439–3447

    Article  CAS  PubMed  Google Scholar 

  31. Chen IJ, Jan Wu YJ, Lin CW et al (2009) Interstitial lung disease in polymyositis and dermatomyositis. Clin Rheumatol 28:639–646

    Article  PubMed  Google Scholar 

  32. Xu Y, Yang CS, Li YJ et al (2016) Predictive factors of rapidly progressive-interstitial lung disease in patients with clinically amyopathic dermatomyositis. Clin Rheumatol 35:113–116

    Article  CAS  PubMed  Google Scholar 

  33. Lu X, Yang H, Shu X et al (2014) Factors predicting malignancy in patients with polymyositis and dermatomyostis: a systematic review and meta-analysis. PLoS ONE 9:e94128

    Article  PubMed  PubMed Central  Google Scholar 

  34. Aguila LA, Lopes MR, Pretti FZ et al (2014) Clinical and laboratory features of overlap syndromes of idiopathic inflammatory myopathies associated with systemic lupus erythematosus, systemic sclerosis, or rheumatoid arthritis. Clin Rheumatol 33:1093–1098

    Article  PubMed  Google Scholar 

  35. Jung M, Bonner A, Hudson M, Baron M, Pope JE (2014) Myopathy is a poor prognostic feature in systemic sclerosis: results from the Canadian scleroderma research group (CSRG) cohort. Scand J Rheumatol 43:217–220

    Article  CAS  PubMed  Google Scholar 

  36. Hochberg MC, Feldman D, Stevens MB (1986) Adult onset polymyositis/dermatomyositis: an analysis of clinical and laboratory features and survival in 76 patients with a review of the literature. Semin Arthritis Rheum 15:168–178

    Article  CAS  PubMed  Google Scholar 

  37. Maugars YM, Berthelot JM, Abbas AA, Mussini JM, Nguyen JM, Prost AM (1996) Long-term prognosis of 69 patients with dermatomyositis or polymyositis. Clin Exp Rheumatol 14:263–274

    CAS  PubMed  Google Scholar 

Download references

Acknowledgements

This research was partially supported by a grant from MSD-SORCOM. We would like to thank all our collaborators who have contributed to the REMICAM cohort. We would also like to thank the Spanish Society of Rheumatology for technical support and the Society of Rheumatology of Madrid (SORCOM) for logistic and human support in this study.

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Authors and Affiliations

  1. Servicio de Reumatología, Hospital Universitario La Paz, Paseo de la Castellana 261, 28046, Madrid, Spain

    Laura Nuño-Nuño

  2. Servicio de Reumatología, Hospital Universitario Doce de Octubre, Madrid, Spain

    Beatriz Esther Joven & Patricia E. Carreira

  3. Servicio de Reumatología, Hospital Universitario Ramón y Cajal, Madrid, Spain

    Valentina Maldonado-Romero & Carmen Larena-Grijalba

  4. Servicio de Reumatología, Hospital Universitario La Princesa, Madrid, Spain

    Irene Llorente Cubas & Eva Gloria Tomero

  5. Servicio de Reumatología, Hospital Universitario Puerta de Hierro, Madrid, Spain

    María Carmen Barbadillo-Mateos

  6. Servicio de Reumatología, Hospital Universitario Madrid Norte Sanchinarro, Madrid, Spain

    Paloma García De la Peña Lefebvre

  7. Servicio de Reumatología, Hospital Universitario Infantil Niño Jesús, Madrid, Spain

    Lucía Ruiz-Gutiérrez & Juan Carlos López-Robledillo

  8. Servicio de Reumatología, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Madrid, Spain

    Henry Moruno-Cruz & Ana Pérez

  9. Servicio de Reumatología, Universidad Europea de Madrid, Hospital Universitario Infanta Sofía, San Sebastián de los Reyes, Madrid, Spain

    Tatiana Cobo-Ibáñez

  10. Servicio de Reumatología, Hospital Universitario Fundación Alcorcón, Alcorcón, Madrid, Spain

    Raquel Almodóvar González

  11. Servicio de Reumatología, Hospital Infanta Leonor, Madrid, Spain

    Leticia Lojo

  12. Instituto de Salud Musculoesquelética, Madrid, Spain

    María Jesús García De Yébenes

  13. Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain

    Francisco Javier López-Longo

Authors
  1. Laura Nuño-Nuño
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  2. Beatriz Esther Joven
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  3. Patricia E. Carreira
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  4. Valentina Maldonado-Romero
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  9. Paloma García De la Peña Lefebvre
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  11. Juan Carlos López-Robledillo
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  12. Henry Moruno-Cruz
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  13. Ana Pérez
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  14. Tatiana Cobo-Ibáñez
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  15. Raquel Almodóvar González
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  16. Leticia Lojo
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  17. María Jesús García De Yébenes
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  18. Francisco Javier López-Longo
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Corresponding author

Correspondence to Laura Nuño-Nuño.

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Ethical approval

This study does not contain any studies with animals performed by any of the authors.

Conflict of interest

Laura Nuño-Nuño has received a research grant from MSD. Francisco Javier López-Longo has received speaker fees from Roche, Bristol-Myers Squibb, UCB and GSK. Beatriz Joven, Patricia Carreira, Valentina Maldonado-Romero, Carmen Larena-Grijalba, Irene Llorente-Cubas, Eva Tomero, María Carmen Barbadillo-Mateos, Paloma García de la Peña Lefebvre, Lucía Ruiz-Gutiérrez, Juan Carlos López-Robledillo, Henry Moruno-Cruz, Ana Pérez-Gómez, Tatiana Cobo-Ibáñez, Raquel Almodóvar, Leticia Lojo-Oliveira, and María Jesús García-de Yébenes declare that they have no conflict of interest.

Informed consent

All procedures performed in studies involving 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 standards. For this type of study formal consent is not required.

Funding

This study was partially funded with a grant from MSD (MSD-SORCOM).

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Nuño-Nuño, L., Joven, B.E., Carreira, P.E. et al. Mortality and prognostic factors in idiopathic inflammatory myositis: a retrospective analysis of a large multicenter cohort of Spain. Rheumatol Int 37, 1853–1861 (2017). https://doi.org/10.1007/s00296-017-3799-x

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  • DOI: https://doi.org/10.1007/s00296-017-3799-x

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