Scleroderma Treatment With Autologous Transplant (STAT) Study

Inclusion Criteria

• Patients with SSc as defined by the American College of Rheumatology with diffuse cutaneous disease (except Group 5) at risk of disease progression
• Patients must have failed a prior >= 4-mponth course of either MMF/Myfortic or cyclophosphamide before being eligible for the study (determined at >= 1 week before start of mobilization); "failure" is defined as evidence of disease progression or absence of improvement; the response prior to MMF of cyclophosphamide will be assessed by the participating site study rheumatologist
• Patients must meet eligibility in at least 1 of the following 6 groups:
• GROUP 1:
• Patients must have 1) both a and b below; and 2) either c, or d
• a) Diffuse cutaneous scleroderma as defined by skin thickening proximal to the elbows and knees and/or involving the torso in addition to distal extremity involvement; a skin score will be obtained but not used to determine eligibility
• b) Duration of systemic sclerosis = 3% neutrophils and/or > 2% eosinophils) from any lavaged lobe
• d) History of SSc-related renal disease that may not be active at the time of screening; stable serum creatinine must be documented for a minimum of 3 months post-renal crisis at the time of the baseline visit; history of scleroderma hypertensive renal crisis is included in this criterion and is defined as follows:
• History of new-onset hypertension based on any of the following (measurements must be repeated and confirmed at least 2 hours apart within 3 days of first event-associated observation, with a change from baseline):
• Systolic blood pressure (SBP) >= 140 mmHg
• Diastolic blood pressure (DBP) >= 90 mmHg
• Rise in SBP >= 30 mmHg compared to baseline
• Rise in DBP >= 20 mmHg compared to baseline
• AND one of the following 5 laboratory criteria:
• Increase of >= 50 % above baseline in serum creatinine
• Proteinuria: >= 2+ by dipstick confirmed by protein:creatinine ratio > 2.5
• Hematuria: >= 2+ by dipstick or > 10 red blood cell (RBC)s/hematopoietic-promoting factor (HPF) (without menstruation)
• Thrombocytopenia: = 70% at screening for the study
• Patients must also have evidence of alveolitis as defined by abnormal chest computed tomography (CT) or bronchoalveolar lavage (BAL)
• GROUP 3: Diffuse scleroderma with disease duration = = 25 plus either
• Erythrocyte sedimentation rate (ESR) > 25 mm/1st hour and/or hemoglobin (Hb) = 30
• GROUP 5:
• Limited cutaneous scleroderma and SSc-related pulmonary disease with FVC 3% neutrophils and/or > 2% eosinophils) from any lavaged lobe
• GROUP 6: Progressive gastrointestinal disease as defined by all of the following items:
• Disease duration of scleroderma = 10% weight loss and on total parenteral nutrition (TPN) or enteral feedings
• High score on distention/ bloating scale (>= 1.60 out of 3.00) on gastrointestinal (GI) questionnaire

Exclusion Criteria

• Subjects with pulmonary, cardiac, hepatic, or renal impairment that would limit their ability to receive cytoreductive therapy and compromise their survival; this includes, but is not restricted to, subjects with any of the following:
• Pulmonary dysfunction defined as:
• Severe pulmonary dysfunction with (1) a hemoglobin corrected DLCO = 45 mmHg without supplemental oxygen, or
• O2 saturation 50 mmHg by resting echocardiogram will require right heart catheterization; if pulmonary artery pressure (PAP) is not evaluable on echocardiogram due to lack of a Tricuspid regurgitant jet, then normal anatomy and function as evidenced by normal right atrium and ventricle size, shape and wall thickness and septum shape must be documented to rule-out PAH; otherwise, right heart catheterization is indicated; prior history of PAH but controlled with medications will not exclude patients from the protocol; PAH is considered controlled with medications if peak systolic pulmonary artery pressure is 2.0 mg/dL; OR
• Active, untreated SSc renal crisis at the time of enrollment; presence of nephrotic range p