Summary:
Systemic sclerosis (SSc) is presumed to be an immune-mediated vasculopathy of unknown etiology. SSc is unresponsive to most immune-modulating therapies except for intravenous cyclophosphamide, which is reported to demonstrate some benefit. We, therefore, dose-escalated cyclophosphamide to 200 mg/kg and added rabbit ATG 7.5 mg/kg along with infusion of unselected hematopoietic stem cells to minimize the cytopenic interval. Engraftment occurred rapidly (day 8) with minimal unexpected toxicity, no infections, and unexpectedly rapid improvement in the modified Rodnan Skin Score.
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References
Herrick AL . Vascular function in systemic sclerosis. Curr Opin Rheumatol 2000; 12: 527–533.
Harvey GR, McHugh NJ . Serologic abnormalities in systemic sclerosis. Curr Opin Rheumatol 1999; 11: 495–502.
Artlett CM, Smith JB, Jimenez SA . Identification of fetal DNA and cells in skin lesions from women with systemic sclerosis. N Engl J Med 1998; 338: 1186–1191.
Medsger Jr TA, Masi AT, Rodnan GP et al. Survival with systemic sclerosis (scleroderma). A life-table analysis of clinical and demographic factors in 309 patients. Ann Intern Med 1971; 75: 369–376.
Binks M, Passweg JR, Furst D et al. Phase I/II trial of autologous stem cell transplantation in systemic sclerosis: procedure related mortality and impact on skin disease. Ann Rheum Dis 2000; 60: 577–584.
McSweeney PA, Nash RA, Sullivan KM et al. High-dose immunosuppressive therapy for severe systemic sclerosis: initial outcomes. Blood 2002; 100: 1602–1610.
Clements PJ, Lachenbruch PA, Seibold JR et al. Skin thickness score in systemic sclerosis: an assessment of interobserver variability in 3 independent studies. J Rheumatol 1993; 20: 1892–1896.
White B, Moore WC, Wigley FM et al. Cyclophosphamide is associated with pulmonary function and survival benefit in patients with scleroderma and alveolitis. Ann Intern Med 2000; 132: 947–954.
O'Dell JR, Steigerwald JC, Kennaugh RC et al. Lack of clinical benefit after treatment of systemic sclerosis with total lymphoid irradiation. J Rheumatol 1989; 16: 1050–1054.
Varga J, Haustein UF, Creech RH et al. Exaggerated radiation-induced fibrosis in patients with systemic sclerosis. JAMA 1991; 265: 3292–3295.
Canadian Association of Radiation Oncologists. Breast radiotherapy after breast-conserving surgery. The Steering Committee on Clinical Practice Guidelines for the Care and Treatment of Breast Cancer. Can Med Assoc J 1998; 158 (Suppl. 3): S35–S42.
Bachleitner-Hofmann T, Machold K, Knobler R et al. Marked and sustained improvement of systemic sclerosis following polychemotherapy for coexistent multiple myeloma. Clin Exp Rheumatol 2002; 20: 85–88.
Coghlan JG, Mukerjee D . The heart and pulmonary vasculature in scleroderma: clinical features and pathobiology. Curr Opin Rheumatol 2001; 13: 495–499.
Tateishi-Yuyama E, Matsubara H, Murohora T et al. Therapeutic angiogenesis for patients with limb ischaemia by autologous transplantation of bone marrow cells: a pilot study and a randomised controlled trial. Lancet 2002; 360: 427–435.
Inaba S, Egashira K, Komori K . Peripheral-blood or bone-marrow mononuclear cells for therapeutic angiogenesis. Lancet 2002; 360: 2083–2084.
Stamm C, Westphal B, Kleine HD et al. Autologous bone-marrow stem-cell transplantation for myocardial regeneration. Lancet 2003; 361: 45–46.
Reyes M, Dudek A, Jahagirdar B et al. Origin of endothelial progenitors in human postnatal bone marrow. J Clin Invest 2002; 109: 337–346.
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Burt, R., Oyama, Y., Traynor, A. et al. Hematopoietic stem cell transplantation for systemic sclerosis with rapid improvement in skin scores: is neoangiogenesis occurring?. Bone Marrow Transplant 32 (Suppl 1), S65–S67 (2003). https://doi.org/10.1038/sj.bmt.1704055
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DOI: https://doi.org/10.1038/sj.bmt.1704055
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