***TASK*** the task is to summarize an input biomedical literature in six sentences 
    ***INPUT*** the input is a biomedical literature 
    ***OUTPUT*** the output is the summary of an input biomedical literature in six sentences 
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    ***EXAMPLES*** 
    Input: More effective therapeutic strategies are required for patients with poor-prognosis systemic sclerosis ( SSc ) . A phase 2 single-arm study of high-dose immunosuppressive therapy ( HDIT ) and autologous CD34-selected hematopoietic cell transplantation ( HCT ) was conducted in 34 patients with diffuse cutaneous SSc .  HDIT included total body irradiation ( 800 cGy ) with lung shielding , cyclophosphamide ( 120 mg/kg ) , and equine antithymocyte globulin ( 90 mg/kg ) . Neutrophil and platelet counts were recovered by 9 ( range , 7 to 13 ) and 11 ( range , 7 to 25 ) days after HCT , respectively . Seventeen of 27 ( 63 % ) evaluable patients who survived at least 1 year after HDIT had sustained responses at a median follow-up of 4 ( range , 1 to 8) years . There was a major improvement in skin ( modified Rodnan skin score , -22.08 ; P < .001 ) and overall function ( modified Health Assessment Question naire Disability Index , -1.03 ; P < .001 ) at final evaluation . Importantly , for the first time , biopsies confirmed a statistically significant decrease of dermal fibrosis compared with baseline ( P < .001 ) . Lung , heart , and kidney function , in general , remained clinical ly stable . There were 12 deaths during the study ( transplantation-related , 8 ; SSc-related , 4 ) . The estimated progression-free survival was 64 % at 5 years . Sustained responses including a decrease in dermal fibrosis were observed exceeding those previously reported with other therapies . HDIT and autologous HCT for SSc should be evaluated in a r and omized clinical trial Autologous hematopoietic stem cell transplantation ( HSCT ) utilizing a myeloablative regimen containing total body irradiation has been performed in patients with systemic sclerosis ( SSc ) , but with substantial toxicity . We , therefore , conducted a phase I non-myeloablative autologous HSCT study in 10 patients with SSc and poor prognostic features . PBSC were mobilized with CY and G-CSF . The PBSC graft was cryopreserved without manipulation and re-infused after the patient was treated with a non-myeloablative conditioning regimen of 200 mg/kg CY and 7.5 mg/kg rabbit antithymocyte globulin . There was a statistically significant improvement of modified Rodnan skin score whereas cardiac ( ejection fraction , pulmonary arterial pressure ) , pulmonary function ( DLCO ) and renal function ( creatinine ) remained stable without significant change . One patient with advanced disease died 2 years after the transplant from progressive disease . After median follow-up of 25.5 months , the overall and progression-free survival rates are 90 and 70 % respectively . Autologous HSCT utilizing a non-myeloablative conditioning regimen appears to result in improved skin flexibility similar to a myeloablative TBI containing regimen , but without the toxicity and risks associated with TBI BACKGROUND Non-r and omised studies of haemopoietic stem-cell transplantation ( HSCT ) in systemic sclerosis have shown improvements in lung function and skin flexibility but high treatment-related mortality . We aim ed to assess safety and efficacy of autologous non-myeloablative HSCT in a phase 2 trial compared with the st and ard of care , cyclophosphamide . METHODS In our open-label , r and omised , controlled phase 2 trial , we consecutively enrolled patients at Northwestern Memorial Hospital ( Chicago , IL , USA ) who were aged younger than 60 years with diffuse systemic sclerosis , modified Rodnan skin scores ( mRSS ) of more than 14 , and internal organ involvement or restricted skin involvement ( mRSS < 14 ) but coexistent pulmonary involvement . We r and omly allocated patients 1:1 by use of a computer-generated sequence with a mixed block design ( blocks of ten and four ) to receive HSCT , 200 mg/kg intravenous cyclophosphamide , and 6·5 mg/kg intravenous rabbit antithymocyte globulin or to receive 1·0 g/m(2 ) intravenous cyclophosphamide once per month for 6 months . The primary outcome for all enrolled patients was improvement at 12 months ' follow-up , defined as a decrease in mRSS ( > 25 % for those with initial mRSS > 14 ) or an increase in forced vital capacity by more than 10 % . Patients in the control group with disease progression ( > 25 % increase in mRSS or decrease of > 10 % in forced vital capacity ) despite treatment with cyclophosphamide could switch to HSCT 12 months after enrolment . This study is registered with Clinical Trials.gov , number NCT00278525 . FINDINGS Between Jan 18 , 2006 , and Nov 10 , 2009 we enrolled 19 patients . All ten patients r and omly allocated to receive HSCT improved at or before 12 months ' follow-up , compared with none of nine allocated to cyclophosphamide ( odds ratio 110 , 95 % CI 14·04-∞ ; p=0·00001 ) . Eight of nine controls had disease progression ( without interval improvement ) compared with no patients treated by HSCT ( p=0·0001 ) , and seven patients switched to HSCT . Compared with baseline , data for 11 patients with follow-up to 2 years after HSCT suggested that improvements in mRSS ( p<0·0001 ) and forced vital capacity ( p<0·03 ) persisted . INTERPRETATION Non-myeloablative autologous HSCT improves skin and pulmonary function in patients with systemic sclerosis for up to 2 years and is preferable to the current st and ard of care , but longer follow-up is needed . FUNDING None OBJECTIVE The aim of this study was to find a new and less cardiotoxic conditioning regimen for high-dose chemotherapy and autologous stem cell transplantation ( aSCT ) in patients with severe SSc and pre-existing cardiac involvement . METHODS Six patients with cardiac involvement were treated for SSc with a conditioning regimen including reduced-dose CYC plus the non-cardiotoxic alkylant thiotepa . All patients received an implantable cardioverter defibrillator ( ICD ) before aSCT . The response at months 6 and 12 was measured according to reduction of the modified Rodnan skin score ( mRSS ) . CT histography was used to monitor pulmonary manifestations , as were echocardiography , N-terminal pro-brain natriuretic peptide ( NT-proBNP ) and troponin for the cardiac involvement . Cardiac events were defined as death or hospitalisation due to heart failure or appropriate discharge of the ICD . RESULTS Between December 2008 and May 2012 , four male and two female patients with a median age of 41 years received aSCT . The median mRSS significantly decreased from 26.5 to 18 and 17.5 at month 6 and 12 , respectively . The total lung volume also significantly improved . Within the median follow-up of 1.6 years ( range 1 - 3.8 ) two patients experienced a relapse of SSc , which results in a progression-free survival rate of 66.6 % . Three patients experienced ICD discharge . CONCLUSION For patients with SSc and cardiac involvement , the use of thiotepa and reduced-dose CYC is feasible and effective . The rate of ICD discharge underlines the need for protection in these endangered patients . This preliminary experience allowed us to use this regimen for our currently recruiting prospect i ve trial ( NCT01895244 ) Objective : Systemic sclerosis ( SSc ) is a generalised autoimmune disease , causing morbidity and a reduced life expectancy , especially in patients with rapidly progressive diffuse cutaneous SSc . As no proven treatment exists , autologous haematopoietic stem cell transplantation ( HSCT ) is employed as a new therapeutic strategy in patients with a poor prognosis . This study reports the effects on survival , skin and major organ function of HSCT in patients with severe diffuse cutaneous SSc . Patients and methods : A total of 26 patients were evaluated .  Peripheral blood stem cells were collected using cyclophosphamide ( 4 g/m2 ) and rHu G-CSF ( 5 to 10 μg/kg/day ) and were reinfused after positive CD34 + selection . For conditioning , cyclophosphamide 200 mg/kg was used . Results : After a median follow-up of 5.3 ( 1–7.5 ) years , 81 % ( n = 21/26 ) of the patients demonstrated a clinical ly beneficial response . The Kaplan – Meier estimated survival at 5 years was 96.2 % ( 95 % CI 89–100 % ) and at 7 years 84.8 % ( 95 % CI 70.2–100 % ) and event-free survival , defined as survival without mortality , relapse or progression of SSc , result ing in major organ dysfunction was 64.3 % ( 95 % CI 47.9–86 % ) at 5 years and 57.1 % ( 95 % CI 39.3–83 % ) at 7 years . Conclusion : This study confirms that autologous HSCT in selected patients with severe diffuse cutaneous SSc results in sustained improvement of skin thickening and stabilisation of organ function up to 7 years after transplantation 
    Output:
    
All the studies showed an improvement in mRSS .
We conclude that ASCT is beneficial in some patients with SSc and that patient selection and conditioning regimens are critical determinants of prognosis and mortality post-ASCT