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Cyclophosphamide treatment for idiopathic inflammatory myopathies and related interstitial lung disease: a systematic review

Clinical Rheumatology volume 34, pages 99–105 (2015)Cite this article

Abstract

The purpose of this study is to review and summarize published information on the use, effectiveness, and adverse effects of cyclophosphamide (CYC) in the management of idiopathic inflammatory myopathies (IIM) and IIM-related interstitial lung disease (IIM-ILD). We performed a systematic search on various databases from May 1975 to May 2014 to find articles concerning CYC therapy in IIM and IIM-ILD. The initial search involved 310 articles, and the 12 articles that met the study criteria were analyzed in detail. All studies were non-randomized. Intravenous CYC (IVCYC) was administered as treatment for IIM in 11 of the studies. Additionally, eight of the twelve studies assessed the effect of CYC in developing resistance steroids or in refractory IIM. IVCYC pulses of 0.3–1.0 g/m2 or 10–30 mg/kg were applied at weekly to monthly intervals for 6–12 months together with either glucocorticoids or another immunosuppressive agent. According to a comprehensive analysis of the studies, 80.8 % (42/52) and 73.1 % (38/52) of patients showed improvement in muscle strength and function. The CK levels of 87.5 % (35/40) of patients fell. The forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLCO) improved in 57.6 % (34/59) and 64.3 % (27/42) of patients. The high-resolution computed tomography (HRCT) findings improved in 67.3 % (35/52) of patients. IVCYC treatment allowed 58.1 % (25/43) of acute/subacute IIM-ILD patients to survive. However, 18 patients died, and the histopathological findings revealed that the 12 deaths were due to diffuse alveolar damage (DAD). HRCT revealed a ground glass (GrG) pattern in 66.7 % (12/18) of the deaths. Of the patients who died, 70 % (7/10) had pneumomediastinum. IVCYC seems to improve both muscle strength and function and lung function in refractory IIM and IIM-ILD patients, and it appears to be relatively well tolerated and safe.

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References

  1. Chen YJ, Wu CY, Shen JL (2007) Predicting factors of interstitial lung disease in dermatomyositis and polymyositis. Acta Derm Venereol 87:33–38

    CAS  PubMed  Article  Google Scholar 

  2. Connors GR, Christopher-Stine L, Oddis CV et al (2010) Interstitial lung disease associated with the idiopathic inflammatory myopathies: what progress has been made in the past 35 years? Chest 138:1464–1474

    PubMed  Article  Google Scholar 

  3. Labirua A, Lundberg IE (2010) Interstitial lung disease and idiopathic inflammatory myopathies: progress and pitfalls. Curr Opin Rheumatol 22:633–638

    PubMed  Article  Google Scholar 

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

    CAS  PubMed  Article  Google Scholar 

  5. Cronin ME, Miller FW, Hicks JE et al (1989) The failure of intravenous cyclophosphamide therapy in refractory idiopathic inflammatory myopathy. J Rheumatol 16:1225–1228

    CAS  PubMed  Google Scholar 

  6. Kuroda H, Morinaga H, Satoh C et al (2003) Clinical study of 10 cases of acute or subacute interstitial pneumonia associated with dermatomyositis. Mod Rheumatol 13:313–318

    PubMed  Article  Google Scholar 

  7. Schnabel A, Reuter M, Biederer J et al (2003) Interstitial lung disease in polymyositis and dermatomyositis: clinical course and response to treatment. Semin Arthritis Rheum 32:273–284

    PubMed  Article  Google Scholar 

  8. Riley P, Maillard SM, Wedderburn LR et al (2004) Intravenous cyclophosphamide pulse therapy in juvenile dermatomyositis. A review of efficacy and safety. Rheumatology 43:491–496

    CAS  PubMed  Article  Google Scholar 

  9. Meyer O, Hayem G, Palazzo E et al (2005) Interstitial lung disease due to polymyositis or dermatomyositis: effect of a 6-month course of i.v. pulse cyclophosphamide. Clin Exp Rheumatol 23:724–724

    CAS  PubMed  Google Scholar 

  10. Kameda H, Nagasawa H, Ogawa H et al (2005) Combination therapy with corticosteroids, cyclosporine A, and intravenous pulse cyclophosphamide for acute/subacute interstitial pneumonia in patients with dermatomyositis. J Rheumatol 32:1719–1726

    CAS  PubMed  Google Scholar 

  11. Yamasaki Y, Yamada H, Yamasaki M et al (2007) Intravenous cyclophosphamide therapy for progressive interstitial pneumonia in patients with polymyositis/dermatomyositis. Rheumatology (Oxford) 46:124–130

    CAS  Article  Google Scholar 

  12. Shi JH, Xu WB, Liu HR et al (2008) Clinico-pathological manifestions in interstitial lung diseases associated with polymyositis-dermatomyositis. Chin J Tuberc Respir Dis 31:250–254

    Google Scholar 

  13. Marie I, Hatron PY, Dominique S et al (2011) Short-term and long-term outcomes of interstitial lung disease in polymyositis and dermatomyositis: a series of 107 patients. Arthritis Rheum 63:3439–3947

    CAS  PubMed  Article  Google Scholar 

  14. Zheng RZ (2012) Clinical analysis of intravenous cyclophosphamide therapy for steroid-resistant polymyositis/dermatomyositis. Dissertation, University of Shandong

  15. Mira-Avendano IC, Parambil JG, Yadav R et al (2013) A retrospective review of clinical features and treatment outcomes in steroid-resistant interstitial lung disease from polymyositis/dermatomyositis. Respir Med 107:890–896

    PubMed  Article  Google Scholar 

  16. Nagappa M, Taly AB, Sinha S et al (2013) Efficacy and limitations of pulse cyclophosphamide therapy in polymyositis and dermatomyositis. J Clin Neuromuscul Dis 14:161–168

    CAS  PubMed  Article  Google Scholar 

  17. American Thoracic Society, European Respiratory Society (2000) Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. Am J Respir Crit Care Med 161:646–664

    Article  Google Scholar 

  18. Ingegnoli F, Lubatti C, Ingegnoli A et al (2012) Interstitial lung disease outcomes by high-resolution computed tomography (HRCT) in Anti-Jo1 antibody-positive polymyositis patients: a single centre study and review of the literature. Autoimmun Rev 11:335–340

    PubMed  Article  Google Scholar 

  19. de Groot K, Dwomoa ADU, Savage CO et al (2001) The value of pulse cyclophosphamide in ANCA-associated vasculitis: meta-analysis and critical review. Nephrol Dial Transplant 16:2018–2027

    PubMed  Article  Google Scholar 

Download references

Funding

This work was supported by the General Program of the National Natural Science Foundation of China (Grant No. 81172860) and by the Science and Technology Commission Foundation of Beijing (No: Z121107001012005).

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Authors and Affiliations

  1. Department of Rheumatology, China-Japan Friendship Hospital, Yinghua East Road, Chaoyang District, 100029, Beijing, China

    Yongpeng Ge, Qinglin Peng, Sigong Zhang, Hang Zhou, Xin Lu & Guochun Wang

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  1. Yongpeng Ge
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  2. Qinglin Peng
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  3. Sigong Zhang
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  4. Hang Zhou
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  5. Xin Lu
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  6. Guochun Wang
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Correspondence to Guochun Wang.

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Ge, Y., Peng, Q., Zhang, S. et al. Cyclophosphamide treatment for idiopathic inflammatory myopathies and related interstitial lung disease: a systematic review. Clin Rheumatol 34, 99–105 (2015). https://doi.org/10.1007/s10067-014-2803-z

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  • DOI: https://doi.org/10.1007/s10067-014-2803-z

Keywords

  • Cyclophosphamide
  • Idiopathic inflammatory myopathy
  • Interstitial lung disease