1. Transplantation For Scleroderma: Data from the Phase I/II Pilot Multicenter Study (FHCRC protocol 1019)

2. TRANSPLANT REGIMEN* TBI 2Gy x 4 ‡ = 8 Gy CTX 60mg/kgx2 - 5 - 4 - 3 -2 -1 0 1 3 5 CD34+ AUTO PBSC DAY G-CSF Equine ATG 15 mg/kg/dose § * PBSC mobilized with G-CSF at 16 ug/kg/d ‡ TBI administered with lung shielding (lung dose= 200 cGy) for last 26 pts § Prednisone with Equine ATG then 0.5 mg/kg/day; day 6-30, taper to day 60 McSweeney, Blood, 2002

3. Patient Characteristics

4. Decrease in Rodnan Skin Score after SCT GEE; p<0.0001 Mean Value Nash, Blood 2007

5. Resolution of Dermal Fibrosis Patient 11 Skin Biopsy A+B : Pre-HCT: grade 5; low + high power C+D : 1 year post-HCT: grade 3; low + high power E+F: 5 years post-HCT: grade 0; low + high power Nash, Blood 2007

6. HEALTH ASSESSMENT (mHAQ)

7. Pulmonary Function after SCT for SSc Forced Vital CapacityDLCO (corrected for Hgb) GEE, p=0.002 Mean Change +2.11, p=.50 GEE, p=0.40 Mean Change -7.07, p=.02

8. Autologous Hematopoietic Cell Transplantation: Survival (n=34) Progression (from SCOT): 1. Respiratory failure- decrease of DLCO >30% or FVC >20%. 2. Renal failure- dialysis or kidney transplantation. 3. Clinical heart failure or LVEF <30%. Progression (from SCOT): 1. Respiratory failure- decrease of DLCO >30% or FVC >20%. 2. Renal failure- dialysis or kidney transplantation. 3. Clinical heart failure or LVEF <30%. 5 yr overall survival= 64% 5 yr PFS= 64%

9. Overall Survival: SCOT-Comparable Patients and Treatment (n=22) n=23 Excluded: No lung shielding -8 Rabbit ATG -1 Eject F. (47%) -1 FVC (27%) -1 Renal failure/PHT-1 3 year OS= 87% 3 year OS= 91% 5 year OS= 78%