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Survival analysis of patients with dermatomyositis and polymyositis

Analysis of 192 Chinese cases

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Abstract

To estimate the mortality rate and identify factors predicting survival in patients with polymyositis (PM) and dermatomyositis (DM). The medical records of 192 PM/DM patients who were treated at Chang Gung Memorial Hospital from 1999 through 2008 were retrospectively reviewed. The Taiwan National Death Registry (1999–2008) was used to obtain their survival status. Thirty-one (16.1%) of the 192 patients with PM/DM had an associated malignancy; 41 (21.4%) had interstitial lung disease (ILD). During the follow-up period, 55 (28.6%) patients died and the overall cumulative survival rate was 79.3% at 1 year, 75.7% at 2 years, 69.9% at 5 years, and 66.2% at 10 years. In univariate analysis, older age at PM/DM onset, anemia, thrombocytopenia, leukopenia, diabetes mellitus, ILD, cancer, and non-use of azathioprine were associated with higher mortality (p = 0.0172, 0.0484, <0.0001, 0.0008, 0.0001, 0.0036, 0.0010, and 0.0019, respectively). In multivariate Cox regression analysis, thrombocytopenia (hazard ratio [HR] 4.94, 95% confidence interval [CI] 2.60–9.37, p < 0.0001), diabetes mellitus (HR 2.57, 95% CI 1.38–4.80, p < 0.0001), cancer (HR 2.30, 95% CI 1.26–4.22, p = 0.0030), and ILD (HR 1.98, 95% CI 1.11–3.51, p = 0.0182) were positively associated with mortality. Use of azathioprine (HR 0.35, 95% CI 0.16–0.74, p = 0.0064) was negatively associated with mortality. This study confirmed the high mortality rate (28.6%) in PM/DM patients. Survival time was significantly reduced in patients with thrombocytopenia, diabetes mellitus, ILD, and cancer patients than in those without these conditions.

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References

  1. Huang YL, Chen YJ, Lin MW et al (2009) Malignancies associated with dermatomyositis and polymyositis in Taiwan: a nationwide population-based study. Br J Dermatol 161:854–560

    Article  PubMed  CAS  Google Scholar 

  2. András 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 

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

    Article  PubMed  CAS  Google Scholar 

  4. Fardet L, Dupuy A, Gain M et al (2009) Factors associated with underlying malignancy in a retrospective cohort of 121 patients with dermatomyositis. Medicine 88:91–97

    Article  PubMed  Google Scholar 

  5. Maoz CR, Langevitz P, Livneh A et al (1998) High incidence of malignancies in patients with dermatomyositis and polymyositis: an 11-year analysis. Semin Arthritis Rheum 27:319–324

    Article  PubMed  CAS  Google Scholar 

  6. 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 

  7. Hirakata M, Nagai S (2000) Interstitial lung disease in polymyositis and dermatomyositis. Curr Opin Rheumatol 12:501–508

    Article  PubMed  CAS  Google Scholar 

  8. Douglas WW, Tazelaar HD, Hartman TE et al (2005) Polymyositis–dermatomyositis associated interstitial lung disease. Am J Respir Crit Care Med 164:1182–1185

    Google Scholar 

  9. Marie I, Hachulla E, Chérin P et al (2002) Interstitial lung disease in polymyositis and dermatomyositis. Arthritis Rheum 47:614–622

    Article  PubMed  CAS  Google Scholar 

  10. Tazelaar HD, Viggiano RW, Pickersgill J et al (1990) Interstitial lung disease in polymyositis and dermatomyositis: clinical features and prognosis as correlated with histologic findings. Am Rev Respir Dis 141:727–733

    Article  PubMed  CAS  Google Scholar 

  11. Fathi M, Lundberg IE (2005) Interstitial lung disease in polymyositis and dermatomyositis. Curr Opin Rheumatol 17:701–706

    Article  PubMed  Google Scholar 

  12. Chen IJ, Tsai WP, Wu YJJ et al (2010) Infections in polymyositis and dermatomyositis: analysis of 192 cases. Rheumatology 49:2429–2437

    Article  PubMed  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  14. Euwer RL, Sontheimer RD (1993) Amyopathic dermatomyositis: a review. J Invest Dermatol 100:124S–127S

    Article  PubMed  CAS  Google Scholar 

  15. Ansell BM (1991) Juvenile dermatomyositis. Rheum Dis Clin North Am 17:931–942

    PubMed  CAS  Google Scholar 

  16. Lu TH, Lee MC, Chou MC (2000) Accuracy of cause-of-death coding in Taiwan: types of miscoding and effects on mortality statistics. Int J Epidemiol 29:336–343

    Article  PubMed  CAS  Google Scholar 

  17. American Thoracic Society/European Respiratory Society International (2002) Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med 165:277–304

    Google Scholar 

  18. Mills ES, Mathews WH (1965) Interstitial pneumonitis in dermatomyositis. J Am Med Assoc 160:1467–1470

    Google Scholar 

  19. 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 

  20. Benbassat J, Gefel D, Larholt K et al (1985) Prognostic factors in polymyositis/dermatomyositis. A computer-assisted analysis of ninety-two cases. Arthritis Rheum 28:249–255

    Article  PubMed  CAS  Google Scholar 

  21. Maugars YM, Berthelot JM, Abbas AA et al (1996) Long-term prognosis of 69 patients with dermatomyositis or polymyositis. Clin Exp Rheumatol 14:263–274

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  24. Ito M, Kaise S, Suzuki S et al (1999) Clinico-laboratory characteristics of patients with dermatomyositis accompanied by rapidly progressive interstitial lung disease. Clin Rheumatol 18:462–427

    Article  PubMed  CAS  Google Scholar 

  25. Dankó K, Ponyi A, Constantin T et al (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 

  26. 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 

  27. Yu KH, Yang CH, Wu YK et al (2006) Diffuse alveolar damage as the initial presentation of dermatomyositis and successfully treated with intravenous immunoglobulin and steroids: a case report. J Musculoskelet Pain 14:45–50

    Article  Google Scholar 

  28. Chen YJ, Wu CY, Shen JL (2001) Predicting factors of malignancy in dermatomyositis and polymyositis: a case-control study. Br J Dermatol 144:825–831

    Article  PubMed  CAS  Google Scholar 

  29. Antiochos BB, Brown LA, Li Z et al (2009) Malignancy is associated with dermatomyositis but not polymyositis in Northern New England, USA. J Rheumatol 36:2704–2710

    Article  PubMed  Google Scholar 

  30. Rozelle A, Trieu S, Chung L (2008) Malignancy in the setting of the anti-synthetase syndrome. J Clin Rheumatol 14:285–288

    Article  PubMed  Google Scholar 

  31. Bronner IM, van der Meulen MF, de Visser M et al (2006) Long-term outcome in polymyositis and dermatomyositis. Ann Rheum Dis 65:1456–1461

    Article  PubMed  CAS  Google Scholar 

  32. Selva-O'Callaghan A, Labrador-Horrillo M, Muñoz-Gall X et al (2005) Polymyositis/dermatomyositis-associated lung disease: analysis of a series of 81 patients. Lupus 14:534–542

    Article  PubMed  Google Scholar 

  33. Hill CL, Zhang Y, Sigurgeirsson B et al (2001) Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. Lancet 357:96–100

    Article  PubMed  CAS  Google Scholar 

  34. Ang P, Sugeng MW, Chua SH (2000) Classical and amyopathic dermatomyositis seen at the National Skin Centre of Singapore: a 3-year retrospective review of their clinical characteristics and association with malignancy. Ann Acad Med Sing 29:219–223

    CAS  Google Scholar 

  35. Kang EH, Lee EB, Shin KC et al (2005) Interstitial lung disease in patients with polymyositis, dermatomyositis and amyopathic dermatomyositis. Rheumatology 44:1282–1286

    Article  PubMed  CAS  Google Scholar 

  36. Fujisawa T, Suda T, Nakamura Y et al (2005) Differences in clinical features and prognosis of interstitial lung diseases between polymyositis and dermatomyositis. J Rheumatol 32:58–64

    PubMed  Google Scholar 

  37. Mielnik P, Wiesik-Szewczyk E, Olesinska M et al (2006) Clinical features and prognosis of patients with idiopathic inflammatory myopathies and anti-Jo-1 antibodies. Autoimmunity 39:243–247

    Article  PubMed  CAS  Google Scholar 

  38. Oddis CV (2000) Current approach to the treatment of polymyositis and dermatomyositis. Curr Opin Rheumatol 12:492–497

    Article  PubMed  CAS  Google Scholar 

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Acknowledgements

The authors are grateful to Chang Gung Memorial Hospital (Project No. CMRPG390211) and the Ministry of Education of Taiwan (Project No. EMRPD180201) for financially supporting this research. This study is based in part on data from the National Health Insurance Research Database provided by the Bureau of National Health Insurance, Department of Health and managed by National Health Research Institutes (Registered number 99067). The interpretation and conclusions contained herein do not represent those of Bureau of National Health Insurance, Department of Health or National Health Research Institutes.

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Correspondence to Kuang-Hui Yu.

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Yu, KH., Wu, YJ.J., Kuo, CF. et al. Survival analysis of patients with dermatomyositis and polymyositis. Clin Rheumatol 30, 1595–1601 (2011). https://doi.org/10.1007/s10067-011-1840-0

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  • DOI: https://doi.org/10.1007/s10067-011-1840-0

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