Treatment of pemphigus vulgaris: part 1 – current therapies

ABSTRACT

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
• clinical outcomes
• cyclophosphamide
• cyclosporine
• immunoadsorption
• IV corticosteroids
• methotrexate
• mycophenolate mofetil
• pemphigus vulgaris
• plasmapheresis
• treatment

Article Highlights

• While many new biologic therapies are being investigated for the treatment of pemphigus, a majority of the world’s population will still be treated with systemic corticosteroids and immunosuppressive agents due to cost and availability.

• Side effects of glucocorticoids are related to cumulative dose, as well as the duration of therapy. It is not known how closely clinicians monitor the cumulative dose of corticosteroids when treating PV patients. This review hopes to encourage clinicians to document the total dose to anticipate and avoid side effects when possible.

• Immunosuppressive agents used as adjuvant therapy in PV such as Mycophenolate mofetil, Azathioprine, Cyclosporine, Methotrexate, and Cyclophosphamide have the common benefit of some level of steroid-sparing effect and decrease of cumulative dose of systemic corticosteroid required. Each agent has unique data on time to remission, duration of remission, and unique risks that can guide or facilitate the choice of agent.

• The patient’s medical condition, comorbidities and overall health, the amount of steroid-sparing effect desired, and data relating to remission and relapse, provided in this review can guide clinicians on their choice of ISA.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This study was supported in part by an educational grant from Dysimmune Diseases Foundation (Plantation, FL, USA).