Rituxan in Churg Strauss Syndrome With Renal Involvement
Condition: Churg-Strauss Syndrome
MeSH terms: Churg-Strauss Syndrome; Syndrome
Intervention: Rituximab; Prednisone
Intervention description: Patients received 4 weekly doses of rituximab 375 mg/m^2.; Prednisone 1 mg/kg/day (not to exceed 80 mg/day) for 4 weeks followed by a taper to 0 mg by 6 months
Intervention MeSH terms: Prednisone; Rituximab
Phase: Phase 2
Study type: Interventional
Primary purpose: Treatment
Allocation: Non-Randomized
Intervention model: Single Group Assignment
Masking: None (Open Label)
Enrollment: 4.0
Sex: All
Minimum age: 18 Years
Healthy volunteers: No
Keywords: Antineutrophil cytoplasmic antibody associated vasculitis; Glomerulonephritis; Rituximab

Summary:

      Churg-Strauss Syndrome (CSS) is a disease characterized by asthma, abnormally high amounts of
      eosinophils (a type of white blood cell), and blood vessel inflammation. About 25% of CSS
      patients develop kidney disease. The goal of this pilot study was to evaluate the safety and
      effectiveness of Rituximab in inducing remission of kidney disease in patients with CSS.
    

Eligibility criteria:

        Inclusion Criteria:

          -  Patients with Churg-Strauss syndrome as defined by meeting one of 3 sets of criteria
             for Churg-Strauss Syndrome described above who have not yet been treated, who have
             failed steroid therapy (partial or non-responders) or who can not been tapered off
             oral prednisone because of documented relapsing disease

          -  Renal involvement (>25% dysmorphic red cell, red blood cell casts, or pauci-immune
             glomerulonephritis on biopsy)

          -  Age >18 years old

          -  Serum creatinine less than or equal to 3.0 mg/dl

          -  Able and willing to give written informed consent and comply with the requirements of
             the study protocol.

          -  Negative serum pregnancy test (for women of child bearing age)

          -  Men and women of reproductive potential must agree to use an acceptable method of
             birth control during treatment and for twelve months after completion of treatment.

        Exclusion Criteria

          -  Severe obstructive or restrictive lung disease (forced expiratory volume in one second
             <1)

          -  Cerebral involvement

          -  Rapidly progressive optic neuropathy or retinal vasculitis

          -  Active gastrointestinal bleeding

          -  Heart failure, including pericarditis or myocarditis.

          -  Hemoglobin <8.5 gm/dL

          -  Platelets <100,000/mm

          -  AST or ALT >2.5 Upper Limit of Normal unless related to primary disease

          -  Positive Hepatitis B or C serology

          -  History of positive HIV testing

          -  Treatment with any investigational agent within 4 weeks of screening or 5 half-lives
             of the investigational drug (whichever is longer)

          -  Previous treatment with Rituximab

          -  History of severe allergic or anaphylactic reactions to humanized or murine monoclonal
             antibodies

          -  History of recurrent significant infection or history of recurrent bacterial
             infections

          -  Known active bacterial, viral fungal mycobacterial, or other infection (including
             tuberculosis or atypical mycobacterial disease, but excluding fungal infections of
             nail beds) or any major episode of infection requiring hospitalization or treatment
             with i.v. antibiotics within 4 weeks of screening or oral antibiotics within 2 weeks
             prior to screening

          -  Lack of peripheral venous access

          -  History of drug, alcohol, or chemical abuse within 6 months prior to screening

          -  Pregnancy or lactation

          -  Concomitant malignancies or previous malignancies, with the exception of adequately
             treated basal or squamous cell carcinoma of the skin or carcinoma in situ of the
             cervix

          -  Significant cardiac or pulmonary disease (including obstructive pulmonary disease)

          -  Any other disease, metabolic dysfunction, physical examination finding, or clinical
             laboratory finding giving reasonable suspicion of a disease or condition that
             contraindicates the use of an investigational drug or that may affect the
             interpretation of the results or render the patient at high risk from treatment
             complications