Autologous Stem Cell Systemic Sclerosis Immune Suppression Trial

Inclusion Criteria: 1. Age 17- 60 years old at the time of pretransplant evaluation 2. An established diagnosis of scleroderma 3. Diffuse cutaneous scleroderma with involvement proximal to the elbow or knee and a Rodnan score (see Appendix V) of \> 14 AND Scleroderma with any one of the following: 1. DLCO \< 80% of predicted or decrease in lung function (DLCO, DLCO/VA or FVC) of 10% or more over 12 months. 2. Pulmonary fibrosis or alveolitis on CT scan or chest X-ray (CXR) (ground glass appearance of alveolitis). 3. Abnormal EKG \[non-specific ST-segment and T-wave (ST-T) (pattern in electrocardiogram) wave abnormalities, low QRS (a pattern seen in an electrocardiogram that indicates the pulses in a heart beat and their duration) voltage, or ventricular hypertrophy\], or pericardial effusion or pericardial enhancement on MRI 4. Gastrointestinal tract involvement confirmed on radiological study. Radiologic findings of scleroderma are small bowel radiographs showing thickened folds with dilated loops, segmentation, and flocculation +/- diverticula, or pseudodiverticula. A hide-bound appearance due to valvulae packing i.e. dilated and crowded circular folds may be present. GI involvement may also be confirmed by D-xylose malabsorption, patulous esophagus on HRCT, or esophageal manometry. OR 4. As published in New England Journal of Medicine (NEJM), 2006, 345:25 2655-2709. Limited or diffuse Systemic Sclerosis with (SSCL) with lung involvement defined as active alveolitis on Bronchoalveolar Lavage (BAL) or ground-glass opacity on CT, a DLCO \< 80% predicted or decrease in lung function (DLCO/VA, DLCO, FVC) of 10% or more in last 12 months. Exclusion Criteria: 1. Significant end organ damage such as: 1. Left Ventricular Function (LVEF) \< 40% on echocardiogram. 2. Untreated life-threatening arrhythmia. 3. Active ischemic heart disease or heart failure. 4. End-stage lung disease characterized by TLC\<45% of predicted value, or DLCO hemoglobin corrected \< 30% predicted . 5. Pulmonary arterial hypertension defined on right heart catheterization as: 1. a resting Mean Pulmonary Artery Pressure (mPAP) \> 25 mmHg; 2. a mPAP \> 30 mmHg following a 500-1000 ml normal saline bolus; 3. pulmonary vascular resistance (PVR) \> 240 dynes\*s/cm5 (\> 3 Wood units) ; or 4. a decrease in cardiac output with fluid challenge (500 - 1000 cc Normal Saline (NS) in 10 minutes) If fluid challenge cannot be done because right atrial (RA) pressure \> 12mm Hg or pulmonary capillary wedge pressure (PCWP) \> 15 m Hg at rest or must be stopped due to safety concerns, patient is excluded as candidate. 6. Serum creatinine \> 1.4 mg/dl. 7. Liver cirrhosis, transaminases \> 3x of normal limits or bilirubin \> 2.0 unless due to Gilbert's disease. 8. Pericardial effusion \> 1 cm on cardiac MRI unless successful pericardiocentesis has been performed 9. Occult or clinical constrictive pericarditis 10. On echocardiogram tricuspid annular peak systolic excursion (TAPSE) ≤ 1.8 cm or, grade II or worse Right Ventricular (RV) or Left Ventricular (LV) diastolic dysfunction 11. On cardiac MRI, a diastolic septal bounce or diastolic septal flattering (D-sign), or diffuse myocardial gadolinium enhancement, or diffuse hypokinesis (patchy late gadolinium myocardial enhancement are not exclusion criteria) 12. Ventricular tachycardia (sustained or non-sustained, multifocal or unifocal) on EKG or 24 hour Holter 2. HIV positive. 3. Uncontrolled diabetes mellitus or any other illness that in the opinion of the investigators would jeopardize the ability of the patient to tolerate aggressive treatment. 4. Prior history of malignancy 5. Positive pregnancy test, inability or unable to pursue effective means of birth control, failure to willingly accept or comprehend irreversible sterility as a side effect of therapy. 6. Psychiatric illness or mental deficiency making compliance with treatment or informed consent impossible. 7. Inability to give informed consent. 8. Major hematological abnormalities such as platelet count \< 100,000/ul or absolute neutrophil count (ANC) \< 1000/ul. 9. Hepatitis B or C positive