Randomized Trial of IVIg With or Without Cyclophosphamide in Pemphigus
Condition: Pemphigus Vulgaris
MeSH terms: Pemphigus
Intervention: intravenous immunoglobulin; cyclophosphamide
Intervention description: Gamunex 10% 500/mg/kg/day x four days per cycle total of four cycles; cyclophosphamide dose of 2mg/kg/day divided into three-times daily oral administration
Intervention MeSH terms: Cyclophosphamide; Immunoglobulins; Immunoglobulins, Intravenous; Antibodies; gamma-Globulins; Rho(D) Immune Globulin
Phase: Phase 2
Study type: Interventional
Primary purpose: Treatment
Allocation: Randomized
Intervention model: Parallel Assignment
Masking: None (Open Label)
Enrollment: 9.0
Sex: All
Minimum age: 18 Years
Maximum age: 85 Years
Healthy volunteers: Accepts Healthy Volunteers
Keywords: pemphigus vulgaris; IVIg; cyclophosphamide; treatment; pemphigus antibodies

Summary:

      The purpose of this study is to compare two standard treatments for pemphigus to determine
      which more effectively improves the clinical manifestations of the disease and decreases
      serum level of the autoantibodies which cause the disease.
    

Eligibility criteria:

        Inclusion Criteria:

          -  Lesions consistent with pemphigus foliaceus or vulgaris

          -  Diagnosis confirmed by histology and IIF ≥ 40 within past month

          -  On ≥20mg/day of prednisone per day for two weeks or ≥ 80mg/day for one week

          -  Women of childbearing potential negative HCG obtained two weeks prior to first IVIg

          -  Agrees to two acceptable forms of contraception* if randomized to cyclophosphamide
             group:

          -  IUD (except progesterone T), Combination oral contraceptives, transdermal patch,
             vaginal ring, hormonal injectables or implantables, male latex condom, diaphragm,
             cervical cap, or vaginal sponge (contains spermicide)

          -  Normal organ function confirmed by CBC, UA, LFTs and Ig levels within defined
             inclusion criteria

          -  Responds yes to at least one of the criteria below:

          -  Persistence of clinical manifestations of disease despite steroid treatment

          -  Flare in disease activity after an attempt at steroid tapering

          -  Failure of established lesions to heal

          -  Rapidly progressive disease.

          -  Conventional therapy is relatively contraindicated i.e. side effects, co-morbid
             conditions

          -  systemic infections, peptic ulcers, osteoporosis, hypertension, cataracts or others

        Exclusion Criteria:

          -  Use of IVIg within past 3 weeks or the use of a cytotoxic drug within the past 2 weeks

          -  Participating in another clinical trial at the time of screening and enrollment

          -  Medical condition that precludes use of IVIg or cyclophosphamide (i.e. pregnancy
             breastfeeding, underlying chronic infection, concurrent opportunistic infection,
             sepsis or volume depletion

          -  Renal insufficiency ( GFR <90, proteinuria (>1+, x 2), creatinine >1.8 or increased
             WBC or RBCs which cannot be explained by cystitis.)

          -  Known hypersensitivity to study drugs, IVIg or cyclophosphamide