Skip to main content

Advertisement

Log in

Early aggressive intra-venous pulse cyclophosphamide therapy for interstitial lung disease in a patient with systemic sclerosis. A case report

  • Case Report
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

Interstitial lung disease is an important cause of mortality and morbidity in patients with systemic sclerosis (SSc). There are currently no recommended guidelines for management of these patients. This is probably due to the rarity of this condition, as well as clinical trials with only a small number of cases. There are published case report and case series along with the two main trials, viz. Scleroderma Lung Study and the Fibrosing Alveolitis Study, but again, there is no consensus on treatment protocols. In this report, we present a case of aggressive interstitial lung disease in a patient with SSc, which improved dramatically on treatment with intra-venous cyclophosphamide and high dose prednisolone therapy.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Tashkin DP, Elashoff R, Clements PJ, Roth MD, Furst DE, Silver RM, Goldin J, Arriola E, Strange C, Bolster MB, Seibold JR, Riley DJ, Hsu VM, Varga J, Schraufnagel D, Theodore A, Simms R, Wise R, Wigley F, White B, Steen V, Read C, Mayes M, Parsley E, Mubarak K, Connolly MK, Golden J, Olman M, Fessler B, Rothfield N, Metersky M, Khanna D, Li N, Li G, Scleroderma Lung Study Research Group (2007) Effects of 1-year treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease. Am J Respir Crit Care Med 176(10):1026–1034. Epub 2007 Aug (Nov 15)

    Article  PubMed  CAS  Google Scholar 

  2. Hoyles RK, Ellis RW, Wellsbury J, Lees B, Newlands P, Goh NS, Roberts C, Desai S, Herrick AL, McHugh NJ, Foley NM, Pearson SB, Emery P, Veale DJ, Denton CP, Wells AU, Black CM, du Bois RM (2006) Arthritis Rheum 54(12):3962–3970. Dec

    Article  PubMed  CAS  Google Scholar 

  3. Clements PJ, Roth MD, Elashoff R, Tashkin DP, Goldin J, Silver RM, Sterz M, Seibold JR, Schraufnagel D, Simms RW, Bolster M, Wise RA, Steen V, Mayes MD, Connelly K, Metersky M, Furst DE, Scleroderma Lung Study Group (2007) Scleroderma lung study (SLS): differences in the presentation and course of patients with limited versus diffuse systemic sclerosis. Ann Rheum Dis 66(12):1641–1647. Epub 2007 May 7 (Dec)

    Article  PubMed  Google Scholar 

  4. Beretta L, Caronni M, Raimondi M, Ponti A, Viscuso T, Origgi L, Scorza R (2007) Oral cyclophosphamide improves pulmonary function in scleroderma patients with fibrosing alveolitis: experience in one centre. Clin Rheumatol 26(2):168–172. Epub 2006 Apr (Feb)

    Article  PubMed  Google Scholar 

  5. Zandman-Goddard G, Tweezer-Zaks N, Shoenfeld Y (2005) New therapeutic strategies for systemic sclerosis–a critical analysis of the literature. Clin Dev Immunol 12(3):165–173. Sep

    Article  PubMed  CAS  Google Scholar 

  6. Kowal-Bielecka O, Kowal K, Rojewska J, Bodzenta-Lukaszyk A, Siergiejko Z, Sierakowska M, Sierakowski S (2005) Cyclophosphamide reduces neutrophilic alveolitis in patients with scleroderma lung disease: a retrospective analysis of serial bronchoalveolar lavage investigations. Ann Rheum Dis 64(9):1343–1346. Sep

    Article  PubMed  CAS  Google Scholar 

  7. Airò P, Danieli E, Parrinello G, Antonioli CM, Cavazzana I, Toniati P, Franceschini F, Cattaneo R (2004) Intravenous cyclophosphamide therapy for systemic sclerosis. A single-center experience and review of the literature with pooled analysis of lung function test results. Clin Exp Rheumatol 22(5):573–578. Sep–Oct

    PubMed  Google Scholar 

  8. Giacomelli R, Valentini G, Salsano F, Cipriani P, Sambo P, Conforti ML, Fulminis A, De Luca A, Farina G, Candela M, Generini S, De Francisci A, Tirri E, Proietti M, Bombardieri S, Gabrielli A, Tonietti G, Cerinic MM (2002) Cyclophosphamide pulse regimen in the treatment of alveolitis in systemic sclerosis. J Rheumatol 29(4):731–736. Apr

    PubMed  CAS  Google Scholar 

  9. Pakas I, Ioannidis JP, Malagari K, Skopouli FN, Moutsopoulos HM, Vlachoyiannopoulos PG (2002) Cyclophosphamide with low or high dose prednisolone for systemic sclerosis lung disease. J Rheumatol 29(2):298–304. Feb

    PubMed  CAS  Google Scholar 

  10. White B, Moore WC, Wigley FM, Xiao HQ, Wise RA (2000) Cyclophosphamide is associated with pulmonary function and survival benefit in patients with scleroderma and alveolitis. Ann Intern Med 132(12):947–954. Jun 20

    PubMed  CAS  Google Scholar 

  11. Davas EM, Peppas C, Maragou M, Alvanou E, Hondros D, Dantis PC (1999) Intravenous cyclophosphamide pulse therapy for the treatment of lung disease associated with scleroderma. Clin Rheumatol 18(6):455–461

    Article  PubMed  CAS  Google Scholar 

  12. Várai G, Earle L, Jimenez SA, Steiner RM, Varga J (1998) A pilot study of intermittent intravenous cyclophosphamide for the treatment of systemic sclerosis associated lung disease. J Rheumatol 25(7):1325–1329. Jul

    PubMed  Google Scholar 

  13. Akesson A, Scheja A, Lundin A, Wollheim FA (1994) Improved pulmonary function in systemic sclerosis after treatment with cyclophosphamide. Arthritis Rheum 37(5):729–735. May

    Article  PubMed  CAS  Google Scholar 

  14. Silver RM, Warrick JH, Kinsella MB, Staudt LS, Baumann MH, Strange C (1993) Cyclophosphamide and low-dose prednisone therapy in patients with systemic sclerosis (scleroderma) with interstitial lung disease. J Rheumatol 20(5):838–844. May

    PubMed  CAS  Google Scholar 

Download references

Disclosures

None

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to R. Peshin.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Peshin, R., O’Neill, L., Browne, P. et al. Early aggressive intra-venous pulse cyclophosphamide therapy for interstitial lung disease in a patient with systemic sclerosis. A case report. Clin Rheumatol 28 (Suppl 1), 31–34 (2009). https://doi.org/10.1007/s10067-009-1098-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-009-1098-y

Keywords

Navigation