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Clinical Rheumatology

, Volume 26, Issue 10, pp 1647–1654 | Cite as

Adult clinically amyopathic dermatomyositis with rapid progressive interstitial lung disease: a retrospective cohort study

  • Shuang YeEmail author
  • Xiao-xiang Chen
  • Xiao-ye Lu
  • Mei-fang Wu
  • Yun Deng
  • Wen-qun Huang
  • Qiang Guo
  • Cheng-de Yang
  • Yue-ying Gu
  • Chun-de Bao
  • Shun-le Chen
Original Article

Abstract

The aim of the study was to investigate the characteristics of adult clinically amyopathic dermatomyositis (CADM) with rapid progressive interstitial lung disease (ILD). Hospitalized patients with dermatomyositis (DM) and polymyositis (PM) between 1998 and 2005 in the Shanghai Renji Hospital were retrospectively studied. One hundred and forty-five patients were classified into CADM, classic DM or PM according to the modified Sontheimer’s definition or Bohan–Peter’s classification criteria. They were further stratified based on the presence or absence of clinical ILD. The Kaplan–Meier survival analysis and COX regression were performed. The predictive factors for ILD and other clinical properties of CADM-ILD were explored. The presence of clinical ILD was a significant risk factor for the poor outcome of DM/PM (OR = 4.237, CI 95%: 1.239–14.49, p = 0.021). Other risk factors are the presence of rashes and elevated urea nitrogen. Patients with DM/PM complicated by ILD had different clinical courses. Patients with CADM-ILD showed a rapidly progressive pattern with 6-month survival rate of 40.8%. The DM-ILD manifested a progressive pattern with a 5-year survival rate of 54%, while PM-ILD was chronic with 5- and 10-year survival rate of 72.4% and 60.3%, respectively. Better preserved muscle strength, elevated erythrocyte sedimentation rate, and hypoalbuminemia may herald ILD in DM/PM. Patients with CADM-ILD who later died had lower PO2, higher lactate dehydrogenase, and prominent arthritis/arthralgia compared with those who survived. The presence of antinuclear antibody seems to be protective. Rapid progressive CADM-ILD is refractory to conventional treatment. ILD is a common complication in over 40% of our hospitalized DM/PM cohort and is also a prominent prognostic indicator. CADM is a special phenotype of DM/PM. CADM-ILD, which is usually rapidly progressive and fatal, requires further investigation.

Keywords

Amyopathic Dermatomyositis Interstitial lung disease 

Notes

Acknowledgment

This work was funded by the Shanghai Leading Academic Discipline Project (T0203) and the Shanghai Health Bureau’s Outstanding Young Medical Professional Program.

References

  1. 1.
    Fathi M, Lundberg IE (2005) Interstitial lung disease in polymyositis and dermatomyositis. Curr Opin Rheumatol 17:701–706PubMedCrossRefGoogle Scholar
  2. 2.
    Takizawa H, Suzuki N, Yanagawa T et al (1996) Outcome in patients with interstitial lung disease and polymyositis–dermatomyositis. A subgroup of poor prognosis. Japanese Journal of Thoracic Diseases 34:1093–1097Google Scholar
  3. 3.
    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 18:462–467PubMedCrossRefGoogle Scholar
  4. 4.
    Lee CS, Chen TL, Tzen CY et al (2002) Idiopathic inflammatory myositis with diffuse alveolar damage. Clin Rheumatol 21:391–396PubMedCrossRefGoogle Scholar
  5. 5.
    Sontheimer RD, Miyagawa S (2003) Potentially fatal interstitial lung disease can occur in clinically amyopathic dermatomyositis. J Am Acad Dermatol 48:797–798PubMedCrossRefGoogle Scholar
  6. 6.
    Kang EH, Lee EB, Shin KC et al (2005) Interstitial lung disease in patients with polymyositis, dermatomyositis and amyopathic dermatomyositis. Rheumatology (Oxford) 44:1282–1286CrossRefGoogle Scholar
  7. 7.
    Neuhaus IM, Connolly MK (2006) An unusual presentation of amyopathic dermatomyositis associated with fatal interstitial lung disease. Arch Dermatol 142:113–114PubMedCrossRefGoogle Scholar
  8. 8.
    Sontheimer RD (1999) Cutaneous features of classic dermatomyositis and amyopathic dermatomyositis. Curr Opin Rheumatol 11:475–482PubMedCrossRefGoogle Scholar
  9. 9.
    Sontheimer RD (2002) Would a new name hasten the acceptance of amyopathic dermatomyositis (dermatomyositis sine myositis) as a distinctive subset within the idiopathic inflammatory dermatomyopathies spectrum of clinical illness? J Am Acad Dermatol 46:626–636PubMedCrossRefGoogle Scholar
  10. 10.
    Bohan A, Peter JB, Bowman RL, Pearson CM (1977) Computer-assisted analysis of 153 patients with polymyositis and dermatomyositis. Medicine 56:255–286PubMedCrossRefGoogle Scholar
  11. 11.
    Santiago MB, Chalhoub M, Pereira ST (1998) Amyopathic dermatomyositis complicated by interstitial pulmonary disease and pneumomediastinum. J Rheumatol 25:2042–2043PubMedGoogle Scholar
  12. 12.
    Yamanishi Y, Maeda H, Konishi F et al (1999) Dermatomyositis associated with rapidly progressive fatal interstitial pneumonitis and pneumomediastinum. Scand J Rheumatol 28:58–61PubMedCrossRefGoogle Scholar
  13. 13.
    Gerami P, Schope JM, McDonald L, Walling HW, Sontheimer RD (2006) A systematic review of adult-onset clinically amyopathic dermatomyositis (dermatomyositis sine’ myositis): a missing link within the spectrum of the idiopathic inflammatory myopathies. J Am Acad Dermatol 54:597–613PubMedCrossRefGoogle Scholar
  14. 14.
    el-Azhary RA, Pakzad SY (2002) Amyopathic dermatomyositis: retrospective review of 37 cases. J Am Acad Dermatol 46:560–565PubMedCrossRefGoogle Scholar
  15. 15.
    Bonnefoy O, Ferretti G, Calaque O et al (2004) Serial chest CT findings in interstitial lung disease associated with polymyositis–dermatomyositis. Eur J Radiol 49:235–244PubMedCrossRefGoogle Scholar
  16. 16.
    Basset-Seguin N, Roujeau JC, Gherardi R, Guillaume JC, Revuz J, Touraine R (1990) Prognostic factors and predictive signs of malignancy in adult dermatomyositis. A study of 32 cases. Arch Dermatol 126:633–637PubMedCrossRefGoogle Scholar
  17. 17.
    Buising KL, Thursky KA, Black JF et al (2006) A prospective comparison of severity scores for identifying patients with severe community acquired pneumonia: reconsidering what is meant by severe pneumonia. Thorax 61:419–424PubMedCrossRefGoogle Scholar
  18. 18.
    Yamasaki Y, Yamada H, Nozaki T et al (2006) Unusually high frequency of autoantibodies to PL-7 associated with milder muscle disease in Japanese patients with polymyositis/dermatomyositis. Arthritis Rheum 54:2004–2009PubMedCrossRefGoogle Scholar
  19. 19.
    Tazelaar HD, Viggiano RW, Pickersgill J, Colby TV (1990) Interstitial lung disease in polymyositis and dermatomyositis. Clinical features and prognosis as correlated with histologic findings. Am Rev Respir Dis 141:727–733PubMedGoogle Scholar
  20. 20.
    Sakamoto N, Mukae H, Fujii T et al (2004) Nonspecific interstitial pneumonia with poor prognosis associated with amyopathic dermatomyositis. Internal Med 43:838–842CrossRefGoogle Scholar
  21. 21.
    Miyazaki E, Ando M, Muramatsu T et al (2005) Early assessment of rapidly progressive interstitial pneumonia associated with amyopathic dermatomyositis. Clin Rheumatol 20:1–4Google Scholar
  22. 22.
    Caproni M, Cardinali C, Parodi A et al (2002) Amyopathic dermatomyositis: a review by the Italian Group of Immunodermatology. Arch Dermatol 138:23–27PubMedCrossRefGoogle Scholar
  23. 23.
    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
  24. 24.
    Fergusson RJ, Davison NM, Nuki G, Crompton GK (1983) Dermatomyositis and rapidly progressive fibrosing alveolitis. Thorax 38:71–72PubMedCrossRefGoogle Scholar
  25. 25.
    Nawata Y, Kurasawa K, Takabayashi K et al (1999) Corticosteroid resistant interstitial pneumonitis in dermatomyositis/polymyositis: prediction and treatment with cyclosporine. J Rheumatol 26:1527–1533PubMedGoogle Scholar
  26. 26.
    Hirakata M, Nagai S (2000) Interstitial lung disease in polymyositis and dermatomyositis. Curr Opin Rheumatol 12:501–508PubMedCrossRefGoogle Scholar
  27. 27.
    Yamasaki Y, Yamada H, Yamasaki M et al (2006) Intravenous cyclophosphamide therapy for progressive interstitial pneumonia in patients with polymyositis/dermatomyositis. Rheumatology (Oxford) 46:124–130CrossRefGoogle Scholar
  28. 28.
    Swigris JJ, Olson AL, Fischer A et al (2006) Mycophenolate mofetil is safe, well tolerated, and preserves lung function in patients with connective tissue disease-related interstitial lung disease. Chest 130:30–36PubMedCrossRefGoogle Scholar
  29. 29.
    Tsukamoto H, Nagafuji K, Horiuchi T et al (2006) A phase I–II trial of autologous peripheral blood stem cell transplantation in the treatment of refractory autoimmune disease. Ann Rheum Dis 65:508–514PubMedCrossRefGoogle Scholar
  30. 30.
    Blumenthal D, Hsiao W (2005) Privatization and its discontents—the evolving Chinese health care system. N Engl J Med 353:1165–1170PubMedCrossRefGoogle Scholar
  31. 31.
    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
  32. 32.
    Sato S, Hirakata M, Kuwana M et al (2005) Autoantibodies to a 140-kd polypeptide, CADM-140, in Japanese patients with clinically amyopathic dermatomyositis. Arthritis Rheum 52:1571–1576PubMedCrossRefGoogle Scholar

Copyright information

© Clinical Rheumatology 2007

Authors and Affiliations

  • Shuang Ye
    • 1
    Email author
  • Xiao-xiang Chen
    • 1
  • Xiao-ye Lu
    • 1
  • Mei-fang Wu
    • 1
  • Yun Deng
    • 1
  • Wen-qun Huang
    • 1
  • Qiang Guo
    • 1
  • Cheng-de Yang
    • 1
  • Yue-ying Gu
    • 1
  • Chun-de Bao
    • 1
  • Shun-le Chen
    • 1
  1. 1.Department of RheumatologyRenji Hospital, Medical School of Shanghai Jiao Tong UniversityShanghaiChina

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