Hematopoietic Stem Cell Support in Vasculitis

Inclusion Criteria:

1. Age 16 to 60 years old at the time of pretransplant evaluation.

2. An established diagnosis of systemic necrotizing vasculitis (Wegener's granulomatosus, polyarteritis nodosum (PAN), allergic angiitis granulomatosus (AAG, also known as Churg Strauss syndrome), microscopic polyangiitis (MPA), or overlap syndrome)Temporal arteritis, or mixed cryoglobulinemia or primary central nervous system vasculitis AND failure of corticosteroids and any of the following at least 6 months of oral or IV cytoxan, rituximab, or cellcept. (Failure defined as: a) patients with a high disease activity and involvement of internal organs as measured by increased FFS > 2 and/or BVAS > 20, or b) patients who develop recurrent flares with subsequent progressive organ damage.)

   OR

   Neurovascular Behcets with recurrent oral and/or genital lesions confirmed by culture to be herpes negative, MRI findings consistent with CNS vasculitis, recurrent neurological symptoms, and clinical confirmation by a Neurologist (e.g., Dr. Rama Gourimeni) AND failure of at least 3 months of oral or IV cytoxan.

   OR

   Pulmonary or neurovascular Sjogrens with positive SSA/SSB confirmed by a rheumatologist and neurologist (if CNS involved) or pulmonologist (if lungs involved) and either recurrent neurologic attacks or progressive pulmonary compromise (dyspnea on exertion, decreased DLCO or CT findings of active disease) despite at least 6 months of intravenous monthly pulse cyclophosphamide.

3. Patient eligibility must be confirmed by two Rheumatologists. For patients with neurovascular Behcets, eligibility need only be confirmed by a neurologist.
4. A minimum CD34+ cell dose of 2.0 x 106/kg post-selection.

Exclusion Criteria:

• 1. Significant end organ damage such as:

  1. LVEF <40% or deterioration of LVEF during exercise test on MUGA or echocardiogram unless due to active disease.
  2. Untreated life-threatening arrhythmia.
  3. Active ischemic heart disease or heart failure.
  4. DLCO < 40% of predicted value unless due to active disease.
  5. Serum creatinine > 2.5 mg/dl, unless due to active disease.

  6. Liver cirrhosis, transaminases >3x of normal limits, or bilirubin >2.0 unless due to Gilberts disease.

     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 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.

     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. Active infection, excluding asymptomatic bacteruria or vaginal candidiasis. 9. Active hepatitis B (HBSAg positive) or active hepatitis C (PCR positive blood lymphocytes).