Inclusion Criteria:

• Age 60 year or < 60 year old at the time of pretransplant evaluation.
• An established diagnosis of scleroderma (125).
• Diffuse cutaneous scleroderma with involvement proximal to the elbow or knee and a Rodnan score (see Appendix V) of > 14 (126)

AND

Scleroderma with any one of the following:

• DLCO < 80% of predicted or decrease in lung function (DLCO, DLCO/VA or FVC) of 10% or more over 12 months.
• Active alveolitis on bronchoalveolar lavage.
• Pulmonary fibrosis or alveolitis on CT scan or CXR (ground glass appearance of alveolitis).
• Renal disease that is not explained by a bacterial infection or other renal disorders. (Subjects must have two or more of the following: proteinuria - greater than trace on dipstick, hematuria - urine blood on dipstick or sediment, hypertension that requires treatment with anti-hypertensive medications or untreated but with a diastolic BP > 95 mm/hg.)
• Abnormal EKG (non-specific ST-T wave abnormalities, low QRS voltage, or ventricular hypertrophy), or pericardial effusion or pericardial enhancement on MRI
• Gastrointestinal tract involvement confirmed on radiological study. Radiologic findings of scleroderma are small bowel radiographs showing thickened folds with dilated loops, segmentation, and flocculation +/- diverticulae, or pseudodiverticulae. 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, or esophageal manometry.

OR

As published in NEJM, 2006, 345:25 2655-2709. Limited or diffuse SSL with lung involvement defined as active alveolitis on 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:

• Poor performance status (ECOG 2) at the time of entry.

• Significant end organ damage such as:

  1. LVEF < 40% or deterioration of LVEF during exercise test on MUGA or 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.
  5. Pulmonary hypertension (systolic pulmonary arterial pressure > 40 mmHg or mean PAP > 25 mmHG measurement by pulmonary arterial catheter).
  6. Serum creatinine > 2.0 mg/dl.
  7. Liver cirrhosis, transaminases > 3x of normal limits or bilirubin > 2.0 unless due to Gilberts disease.
  8. Pericardial effusion> 200ml unless successful pericardiacentesis
  9. Tricuspid annular peak systolic excursion (TAPSE) ≤ 1.9 cm
  10. MRI of heart showing D sign (intraventricular flattering)
• HIV positive.
• 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.
• Prior history of malignancy except localized basal cell or squamous skin cancer. Other malignancies for which the patient is judged to be cured by local surgical therapy, such as (but not limited to) head and neck cancer, or stage I or II breast cancer will be considered on an individual basis.
• 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.
• Psychiatric illness or mental deficiency making compliance with treatment or informed consent impossible.
• Inability to give informed consent.
• Major hematological abnormalities such as platelet count < 100,000/ul or ANC < 1000/ul.
• Patients with duration of disease > 5 years.
• Exclude if > than 6 prior monthly IV cyclophosphamide treatments.