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Allogeneic marrow transplantation in patients with severe systemic sclerosis: resolution of dermal fibrosis.

Publication ,  Journal Article
Nash, RA; McSweeney, PA; Nelson, JL; Wener, M; Georges, GE; Langston, AA; Shulman, H; Sullivan, KM; Lee, J; Henstorf, G; Storb, R; Furst, DE
Published in: Arthritis Rheum
June 2006

OBJECTIVE: To evaluate the safety and efficacy of allogeneic hematopoietic cell transplantation (HCT) after myeloablative conditioning in patients with severe systemic sclerosis (SSc). METHODS: Eligibility criteria for the study included SSc patients with features indicative of a poor prognosis. The myeloablative conditioning regimen included busulfan, cyclophosphamide, and antithymocyte globulin. Prophylaxis for graft-versus-host disease (GVHD) consisted of cyclosporine and methotrexate. Bone marrow was transplanted from HLA-identical siblings. RESULTS: Two patients with diffuse cutaneous SSc and lung involvement who were refractory to conventional immunosuppressive treatment were enrolled in the study. In patient 1, there were no complications related to the conditioning regimen, and GVHD did not develop after transplantation. At 5 years after HCT, there was nearly complete resolution of the scleroderma and marked improvement in physical functioning. Internal organ function improved (lung) or remained stable. On examination of serial skin biopsy samples, there was resolution of the dermal fibrosis. Patient 2 experienced skin toxicity from the conditioning regimen and hypertensive crisis that was likely related to high-dose corticosteroids given for treatment of GVHD. Although this patient experienced an improvement in scleroderma and overall functioning, a fatal opportunistic infection developed 17 months after HCT. CONCLUSION: Allogeneic HCT may result in sustained remission of SSc. GVHD and opportunistic infections are the major risks associated with allogeneic HCT for SSc, as for allogeneic HCT in general.

Duke Scholars

Published In

Arthritis Rheum

DOI

ISSN

0004-3591

Publication Date

June 2006

Volume

54

Issue

6

Start / End Page

1982 / 1986

Location

United States

Related Subject Headings

  • Transplantation, Homologous
  • Transplantation Conditioning
  • Skin
  • Scleroderma, Systemic
  • Myeloablative Agonists
  • Methotrexate
  • Immunosuppressive Agents
  • Humans
  • Hematopoietic Stem Cell Transplantation
  • Graft vs Host Disease
 

Citation

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Nash, R. A., McSweeney, P. A., Nelson, J. L., Wener, M., Georges, G. E., Langston, A. A., … Furst, D. E. (2006). Allogeneic marrow transplantation in patients with severe systemic sclerosis: resolution of dermal fibrosis. Arthritis Rheum, 54(6), 1982–1986. https://doi.org/10.1002/art.21908
Nash, Richard A., Peter A. McSweeney, J Lee Nelson, Mark Wener, George E. Georges, Amelia A. Langston, Howard Shulman, et al. “Allogeneic marrow transplantation in patients with severe systemic sclerosis: resolution of dermal fibrosis.Arthritis Rheum 54, no. 6 (June 2006): 1982–86. https://doi.org/10.1002/art.21908.
Nash RA, McSweeney PA, Nelson JL, Wener M, Georges GE, Langston AA, et al. Allogeneic marrow transplantation in patients with severe systemic sclerosis: resolution of dermal fibrosis. Arthritis Rheum. 2006 Jun;54(6):1982–6.
Nash, Richard A., et al. “Allogeneic marrow transplantation in patients with severe systemic sclerosis: resolution of dermal fibrosis.Arthritis Rheum, vol. 54, no. 6, June 2006, pp. 1982–86. Pubmed, doi:10.1002/art.21908.
Nash RA, McSweeney PA, Nelson JL, Wener M, Georges GE, Langston AA, Shulman H, Sullivan KM, Lee J, Henstorf G, Storb R, Furst DE. Allogeneic marrow transplantation in patients with severe systemic sclerosis: resolution of dermal fibrosis. Arthritis Rheum. 2006 Jun;54(6):1982–1986.
Journal cover image

Published In

Arthritis Rheum

DOI

ISSN

0004-3591

Publication Date

June 2006

Volume

54

Issue

6

Start / End Page

1982 / 1986

Location

United States

Related Subject Headings

  • Transplantation, Homologous
  • Transplantation Conditioning
  • Skin
  • Scleroderma, Systemic
  • Myeloablative Agonists
  • Methotrexate
  • Immunosuppressive Agents
  • Humans
  • Hematopoietic Stem Cell Transplantation
  • Graft vs Host Disease