Autologous stem cells transplantation in patient treated for systematic sclerodermia with refraktory interstitial pulmonary process
Abstract
Lung involvement in its interstitial and vascular form represents a serious manifestation of systemic sclerosis (SSc) and often determines the 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 transplantation EBMT/EULAR je SSc.
A case of 57-years old SSc patient with a severe intersitial lung involvement is described. Despite of repeated infusions of cyclophosphamide combinated with corticosteroids progresion of lung fibrosis and active alveolitis with a desrease of DLCO were observed.
Therefore the patient was indicated to immunoablative treatment followed by autologous stem cell transplantation. The procedure was complicated by a masive lung hemorrhage but later regression of alevolitis according to HRCT scan was observed., Eight monts later patient died because of acute myocardial infarction.
This is the first described case of SSc treated with stem transplant in the Czech Republic.