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Clinical Rheumatology

, Volume 30, Issue 12, pp 1595–1601 | Cite as

Survival analysis of patients with dermatomyositis and polymyositis

Analysis of 192 Chinese cases
  • Kuang-Hui Yu
  • Yeong-Jian Jan Wu
  • Chang-Fu Kuo
  • Lai-Chu See
  • Yu-Ming Shen
  • Hsiao-Chun Chang
  • Shue-Fen Luo
  • Huei-Huang Ho
  • I-Jung Chen
Original Article

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.

Keywords

Dermatomyositis Polymyositis Prognosis Survival analysis 

Notes

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.

Disclosures

None.

References

  1. 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 161:854–560PubMedCrossRefGoogle Scholar
  2. 2.
    András C, Ponyi A, Constantin T et al (2008) Dermatomyositis and polymyositis associated with malignancy: a 21-year retrospective study. J Rheumatol 35:438–444PubMedGoogle Scholar
  3. 3.
    Airio A, Kautiainen H, Hakala M (2006) Prognosis and mortality of polymyositis and dermatomyositis patients. Clin Rheumatol 25:234–239PubMedCrossRefGoogle Scholar
  4. 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 88:91–97PubMedCrossRefGoogle Scholar
  5. 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 27:319–324PubMedCrossRefGoogle Scholar
  6. 6.
    Chen IJ, Jan Wu YJ, Lin CW et al (2009) Interstitial lung disease in polymyositis and dermatomyositis. Clin Rheumatol 28:639–646PubMedCrossRefGoogle Scholar
  7. 7.
    Hirakata M, Nagai S (2000) Interstitial lung disease in polymyositis and dermatomyositis. Curr Opin Rheumatol 12:501–508PubMedCrossRefGoogle Scholar
  8. 8.
    Douglas WW, Tazelaar HD, Hartman TE et al (2005) Polymyositis–dermatomyositis associated interstitial lung disease. Am J Respir Crit Care Med 164:1182–1185Google Scholar
  9. 9.
    Marie I, Hachulla E, Chérin P et al (2002) Interstitial lung disease in polymyositis and dermatomyositis. Arthritis Rheum 47:614–622PubMedCrossRefGoogle Scholar
  10. 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 141:727–733PubMedCrossRefGoogle Scholar
  11. 11.
    Fathi M, Lundberg IE (2005) Interstitial lung disease in polymyositis and dermatomyositis. Curr Opin Rheumatol 17:701–706PubMedCrossRefGoogle Scholar
  12. 12.
    Chen IJ, Tsai WP, Wu YJJ et al (2010) Infections in polymyositis and dermatomyositis: analysis of 192 cases. Rheumatology 49:2429–2437PubMedCrossRefGoogle Scholar
  13. 13.
    Bohan A, Peter JB (1975) Polymyositis and dermatomyositis (first of two parts). N Engl J Med 292:344–347PubMedCrossRefGoogle Scholar
  14. 14.
    Euwer RL, Sontheimer RD (1993) Amyopathic dermatomyositis: a review. J Invest Dermatol 100:124S–127SPubMedCrossRefGoogle Scholar
  15. 15.
    Ansell BM (1991) Juvenile dermatomyositis. Rheum Dis Clin North Am 17:931–942PubMedGoogle Scholar
  16. 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 29:336–343PubMedCrossRefGoogle Scholar
  17. 17.
    American Thoracic Society/European Respiratory Society International (2002) Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med 165:277–304Google Scholar
  18. 18.
    Mills ES, Mathews WH (1965) Interstitial pneumonitis in dermatomyositis. J Am Med Assoc 160:1467–1470Google Scholar
  19. 19.
    Medsger TA Jr, Robinson H, Masi AT (1971) Factors affecting survivorship in polymyositis. A life-table study of 124 patients. Arthritis Rheum 14:249–258PubMedCrossRefGoogle Scholar
  20. 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 28:249–255PubMedCrossRefGoogle Scholar
  21. 21.
    Maugars YM, Berthelot JM, Abbas AA et al (1996) Long-term prognosis of 69 patients with dermatomyositis or polymyositis. Clin Exp Rheumatol 14:263–274PubMedGoogle Scholar
  22. 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 28:2230–2237PubMedGoogle Scholar
  23. 23.
    Sultan SM, Ioannou Y, Moss K et al (2002) Outcome in patients with idiopathic inflammatory myositis: morbidity and mortality. Rheumatology 41:22–26PubMedCrossRefGoogle Scholar
  24. 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 18:462–427PubMedCrossRefGoogle Scholar
  25. 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) 83:35–42CrossRefGoogle Scholar
  26. 26.
    Torres C, Belmonte R, Carmona L et al (2006) Survival, mortality and causes of death in inflammatory myopathies. Autoimmunity 39:205–215PubMedCrossRefGoogle Scholar
  27. 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 14:45–50CrossRefGoogle Scholar
  28. 28.
    Chen YJ, Wu CY, Shen JL (2001) Predicting factors of malignancy in dermatomyositis and polymyositis: a case-control study. Br J Dermatol 144:825–831PubMedCrossRefGoogle Scholar
  29. 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 36:2704–2710PubMedCrossRefGoogle Scholar
  30. 30.
    Rozelle A, Trieu S, Chung L (2008) Malignancy in the setting of the anti-synthetase syndrome. J Clin Rheumatol 14:285–288PubMedCrossRefGoogle Scholar
  31. 31.
    Bronner IM, van der Meulen MF, de Visser M et al (2006) Long-term outcome in polymyositis and dermatomyositis. Ann Rheum Dis 65:1456–1461PubMedCrossRefGoogle Scholar
  32. 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 14:534–542PubMedCrossRefGoogle Scholar
  33. 33.
    Hill CL, Zhang Y, Sigurgeirsson B et al (2001) Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. Lancet 357:96–100PubMedCrossRefGoogle Scholar
  34. 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 29:219–223Google Scholar
  35. 35.
    Kang EH, Lee EB, Shin KC et al (2005) Interstitial lung disease in patients with polymyositis, dermatomyositis and amyopathic dermatomyositis. Rheumatology 44:1282–1286PubMedCrossRefGoogle Scholar
  36. 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 32:58–64PubMedGoogle Scholar
  37. 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 39:243–247PubMedCrossRefGoogle Scholar
  38. 38.
    Oddis CV (2000) Current approach to the treatment of polymyositis and dermatomyositis. Curr Opin Rheumatol 12:492–497PubMedCrossRefGoogle Scholar

Copyright information

© Clinical Rheumatology 2011

Authors and Affiliations

  • Kuang-Hui Yu
    • 1
    • 2
  • Yeong-Jian Jan Wu
    • 1
    • 2
  • Chang-Fu Kuo
    • 1
    • 2
  • Lai-Chu See
    • 3
  • Yu-Ming Shen
    • 4
  • Hsiao-Chun Chang
    • 1
  • Shue-Fen Luo
    • 1
    • 2
  • Huei-Huang Ho
    • 1
    • 2
  • I-Jung Chen
    • 2
    • 5
  1. 1.Division of Allergy, Immunology and RheumatologyChang Gung Memorial HospitalTao-YuanTaiwan
  2. 2.Chang Gung UniversityTao-YuanTaiwan
  3. 3.Department of Public HealthCollege of Medicine, and Biostatistics Core Laboratory, Molecular Medicine Research Center, Chang Gung UniversityTao-YuanTaiwan
  4. 4.Biostatistics Consulting Center, Department of Public HealthChang Gung UniversityTao-YuanTaiwan
  5. 5.School of MedicineChang Gung UniversityTao-YuanTaiwan

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