Treatment of pemphigus vulgaris: part 1 - current therapies

Expert Rev Clin Immunol. 2019 Oct;15(10):1047-1060. doi: 10.1080/1744666X.2020.1672535. Epub 2019 Oct 10.


Introduction: While biologic agents that can be used for treating pemphigus vulgaris (PV) are increasing, themajority of the world's PV patients can afford only corticosteroids (CS) and some immunosuppressive agents (ISA). Areas covered: The spectrum of side effects encountered when PV patients receive high-dose, long-term CS and ISA are presented based on total dose and duration of therapy. The steroid-sparing effect of individual ISA as documented in published studies and their clinical outcomes, in terms of duration of remissions, frequency of relapses and time to relapse, are presented, so that comparisons are possible. Thus, rational choices can be made for the individual patient. Expert opinion: In 2019, the majority of PV patients globally will continue to be treated with CS and ISA. This review will help clinicians and patients become aware of when to anticipate which side effects and if possible, to prevent or avoid them. It provides guidelines to maximize the clinical benefits of ISA in inducing and maintaining remission and minimizing side effects by monitoring them.

Keywords: Azathioprine; IV corticosteroids; clinical outcomes; cyclophosphamide; cyclosporine; immunoadsorption; methotrexate; mycophenolate mofetil; pemphigus vulgaris; plasmapheresis; treatment.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adrenal Cortex Hormones / adverse effects
  • Adrenal Cortex Hormones / therapeutic use
  • Fractures, Bone / chemically induced
  • Humans
  • Hypothalamo-Hypophyseal System / drug effects
  • Immunosuppressive Agents / adverse effects
  • Immunosuppressive Agents / therapeutic use
  • Muscular Diseases / chemically induced
  • Pemphigus / drug therapy*
  • Pituitary ACTH Hypersecretion / chemically induced
  • Pituitary-Adrenal System / drug effects
  • Plasmapheresis


  • Adrenal Cortex Hormones
  • Immunosuppressive Agents