title: Intravenous immunoglobulin therapy for patients with pemphigus foliaceus unresponsive to conventional therapy.
abstract: BACKGROUND
  Pemphigus foliaceus (PF) is a chronic autoimmune blistering skin disease that is commonly treated with oral corticosteroids and immunosuppressive therapy. In some patients, PF can be refractory to treatment and the resultant side effects of prolonged immune suppression can be potentially fatal. Alternative therapies are needed.
  
  
  OBJECTIVE
  The purpose of this study is to report treatment outcomes with IVIg therapy in 11 patients with severe PF refractory to prednisone and other immunosuppressive therapy.
  
  
  METHODS
  Selection criteria included documentation of a biopsy and immunopathology in 11 patients who were resistant to treatment or experienced side effects to conventional therapy. IVIg was administered according to a defined protocol. The parameters used to assess clinical response to IVIg included time observed for effective control of disease, duration of IVIg maintenance therapy, total duration of IVIg, number of IVIg cycles, systemic drug therapy, and the frequency of recurrences and relapses. The pre-IVIg and post-IVIg data were statistically analyzed by means of the SAS UNIVARIATE and 2-sided Wilcoxon sign rank and sign tests.
  
  
  RESULTS
  All patients had an effective clinical response and remained in clinical remission for a mean period of 18.6 months after discontinuation of IVIg therapy. Serious side effects from IVIg use were not observed.
  
  
  CONCLUSIONS
  IVIg therapy appears to have potential as a biologic alternative agent in inducing and maintaining clinical remissions in patients with PF who are resistant to more standard conventional treatment. IVIg is effective as monotherapy and may be needed for a period of several months to achieve a long-term clinical remission.
authors.affiliation: Department of Medicine, New England Baptist Hospital, and Harvard School of Dental Medicine, Boston, Massachusetts 02115, USA.
authors.forename: A Razzaque
authors.initials: AR
authors.lastname: Ahmed
authors.forename: Naveed
authors.initials: N
authors.lastname: Sami
mesh_terms: D000328:Adult; D000368:Aged; D004334:Drug Administration Schedule; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000188:drug therapy* / Q000473:pathology; D011788:Quality of Life; D012008:Recurrence; D016896:Treatment Outcome
chemical_list: D016756:Immunoglobulins, Intravenous