title: IVIG therapy in pemphigus vulgaris has corticosteroid-sparing and immunomodulatory effects.
abstract: BACKGROUND
  Intravenous immunoglobulin (IVIG) is a biological agent composed of polyclonal antibodies prepared from a large cohort of human plasma pools. IVIG is increasingly used for the treatment of various antibody-mediated diseases, including pemphigus vulgaris (PV).
  
  
  OBJECTIVE
  The aim of the study was to evaluate the benefit and safety profiles of high dose IVIG therapy in PV patients determined by clinical remission, corticosteroid-sparing and immunomodulatory effects, and adverse events at 12 months' follow up.
  
  
  METHODS
  Ten PV patients underwent 3-8 monthly cycles of IVIG therapy at a dose of 2 g/kg per cycle. The pemphigus disease area index (PDAI) score, direct immunofluorescence (DIF), indirect immunofluorescence (IIF), and corticosteroid dosage were evaluated before IVIG therapy, after each cycle, and at 6 and 12 months' follow up.
  
  
  RESULTS
  The baseline PDAI score was 75.70 ± 21.0 and baseline prednisone dosage was 201.60 ± 71.7 mg/day. The PDAI score reduction of 98% was achieved at 12 months' follow up and a corticosteroid dose reduction of 90% corresponded to clinical improvement. The decrease in both values was statistically significant (P = 0.002, respectively). At 12 months' follow up, seven patients were shown to be negative on IIF, of whom three proved to be negative on DIF. Adverse events were mild and transient and did not require the cessation of IVIG therapy.
  
  
  CONCLUSIONS
  IVIG induced long-term clinical remission, while displaying a corticocorticosteroid-sparing effect and evoking a long-standing immunomodulatory effect in PV patients. The safety profile of IVIG therapy was assessed as good.
authors.affiliation: Department of Dermatovenerology, Faculty of Medicine, Comenius University, University Hospital, Bratislava, Slovakia.
authors.forename: Danka
authors.initials: D
authors.lastname: Svecova
mesh_terms: D000293:Adolescent; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D056747:Immunomodulation / Q000187:drug effects*; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000188:drug therapy*; D011241:Prednisone / Q000008:administration & dosage; D012720:Severity of Illness Index; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000893:Anti-Inflammatory Agents; D016756:Immunoglobulins, Intravenous; D011241:Prednisone
keywords: autoimmune bullous disease; corticosteroid-sparing effect; immunomodulatory effect; intravenous immunoglobulin (IVIG); pemphigus vulgaris; safety profile