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CZECH MEDICAL ASSOCIATION J. Ev. PURKYNĚ |
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Česky / Czech version | Čes. Revmatol., 13, 2005, No. 2, p. 73–76. |
Autologous stem cell transplantation in a systemic sclerosis patient with refractory interstitial
lung involvement Bečvář, R., Kozák T.1, Forejtová Š., Slabý J.1, Bartáková H.2, Kolář M.3, Pachl J.3 Revmatologický ústav, Praha 1Oddělení klinické hematologie FNKV, Praha2II. interní klinika FNKV, Praha3Klinika anesteziologie a resuscitace FNKV, Praha |
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Summary: Lung involvement in its interstitial and vascular form represents a serious manifestation of systemic
sclerosis (SSc) and often determines the disease prognosis. As the most severe forms of SSc
are often refractory to conventional immunosuppressive therapy, SSc is the second most common
condition indicated to immunoablative treatment in the European registry of autologous bone
marrow transplantations EBMT/EULAR je SSc. A case of 57-years old SSc patient with a severe
interstitial lung involvement is described. Despite of repeated infusions of cyclophosphamide
combined with corticosteroids progression of lung fibrosis and active alveolitis with a decrease
of DLCO were observed. Therefore the patient was indicated to immunoablative treatment followed
by autologous stem cell transplantation. The procedure was complicated by a massive lung
hemorrhage but later regression of alevolitis according to HRCT scan was observed. Eight
months later the patient died because of acute myocardial infarction. This is the first described
case of SSc treated with stem cell transplant in the Czech Republic.
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