Skip to main content

Advertisement

Log in

Long-term follow-up after nonmyeloablative allogeneic hematopoietic stem cell transplantation for systemic sclerosis

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

Abstract

Systemic sclerosis (SSc) characteristically consists of fibrotic changes in various organs, and immunological abnormality is the main cause of the disease. Although high-dose immunosuppressive therapies with autologous or allogeneic hematopoietic stem cell support can reverse the disease course, they have a high treatment-related mortality. We report the successful use of nonmyeloablative allogeneic hematopoietic stem cell transplantation (HSCT) for SSc. A 40-year-old woman with diffuse scleroderma and interstitial pneumonia underwent allogeneic peripheral blood stem cell transplantation from an HLA-identical sibling after conditioning with low-dose total-body irradiation and fludarabine. Prophylaxis for graft-versus-host disease (GVHD) consisted of cyclosporine and mycophenolate mofetil. No infection or acute GVHD developed. One year after transplantation, the patient developed membranous glomerulopathy caused by chronic GVHD that was successfully treated with prednisolone. The patient’s skin score decreased dramatically, and her pulmonary function is stable 4 years after transplantation. Nonmyeloablative allogeneic HSCT may be more effective than conventional therapies for SSc.

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. Furst DE (2000) Rational therapy in the treatment of systemic sclerosis. Curr Opin Rheumatol 12:540–544

    Article  PubMed  CAS  Google Scholar 

  2. Burt RK, Marmont A, Oyama Y, Slavin S, Arnold R, Hiepe F et al (2006) Randomized controlled trials of autologous hematopoietic stem cell transplantation for autoimmune diseases: The evolution from myeloablative to lymphoablative transplant regimens. Arthritis Rheum 54:3750–3760

    Article  PubMed  Google Scholar 

  3. Nash RA, McSweeney PA, Crofford LJ, Abidi M, Chen CS, Godwin JD et al (2007) High-dose immunosuppressive therapy and autologous hematopoietic cell transplantation for severe systemic sclerosis: long-term follow-up of the U.S. multicenter pilot study. Blood 110:1388–1396

    Article  PubMed  CAS  Google Scholar 

  4. Farge D, Passweg J, van Laar JM, Marjanovic Z, Besenthal C, Finke J et al (2004) Autologous stem cell transplantation in the treatment of systemic sclerosis: report from the EBMT/EULAR registry. Ann Rheum Dis 63:974–981

    Article  PubMed  CAS  Google Scholar 

  5. Nash RA, McSweeney PA, Nelson JL, Wener M, Georges GE, Langston AA et al (2006) Allogeneic marrow transplantation in patients with severe systemic sclerosis. Arthritis Rheum 54:1982–1986

    Article  PubMed  Google Scholar 

  6. Sandmaier BM, Mackinnon S, Childs RW (2007) Reduced intensity conditioning for allogeneic hematopoietic cell transplantation: current perspectives. Biol Blood Marrow Transplant 13:87–97

    Article  PubMed  Google Scholar 

  7. Mielcarek M, Martin PJ, Leisenring W, Flowers MED, Maloney DG, Sandmaier BM et al (2003) Graft-versus-host disease after nonmyeloablative versus conventional hematopoietic stem cell transplantation. Blood 102:756–762

    Article  PubMed  CAS  Google Scholar 

  8. Loh Y, Oyama Y, Statkute L, Verda L, Quigley K, Yaung K et al (2007) Non-myeloablative allogeneic hematopoietic stem cell transplantation for severe systemic sclerosis: graft-versus-autoimmunity without graft-versus-host disease? Bone Marrow Transplant 39:435–437

    Article  PubMed  CAS  Google Scholar 

  9. Lin J, Markowitz GS, Nicolaides M, Hesdorffer CS, Appel GB, D’Agati VD (2001) Membranous glomerulopathy associated with graft-versus-host disease following allogeneic stem cell transplantation. Am J Nephrol 21:351–356

    Article  PubMed  CAS  Google Scholar 

  10. Wielosz E, Majdan M, Suszek D, Smarz-Widelska I, Korolczuk A, Korobowicz E (2007) Nephrotic syndrome as a clinical manifestation of systemic sclerosis. Rheumatol Int 27:1087–1089

    Article  PubMed  Google Scholar 

  11. Korbling M, Anderlini P (2001) Peripheral blood stem cell versus bone marrow allotransplantation: does the source of hematopoietic stem cells matter? Blood 98:2900–2908

    Article  PubMed  CAS  Google Scholar 

  12. Zaucha JM, Zellmer E, Georges G, Little MT, Storb R, Storer B et al (2001) G-CSF-mobilized peripheral blood mononuclear cells added to marrow facilitates engraftment in nonmyeloablated canine recipients: CD3 cells are required. Biol Blood Marrow Transplant 7:613–619

    Article  PubMed  CAS  Google Scholar 

Download references

Disclosures

None disclosed.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Junji Nishimura.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Shiratsuchi, M., Motomura, S., Abe, Y. et al. Long-term follow-up after nonmyeloablative allogeneic hematopoietic stem cell transplantation for systemic sclerosis. Clin Rheumatol 27, 1207–1209 (2008). https://doi.org/10.1007/s10067-008-0927-8

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-008-0927-8

Keywords

Navigation