Rituximab in the treatment of pemphigus

ABSTRACT

Introduction: Pemphigus vulgaris is an autoimmune mucocutaneous blistering disease with a high morbidity and mortality rate. Autoantibodies against molecules that bind keratinocytes to each other in the skin or mucosa leads to cell separation known as acantholysis. Flaccid blisters and erosions are characteristic clinical representations. The disease could be controlled with conventional therapy consisting of systemic corticosteroids and immunosuppressive agents. Long term use of these agents can lead to severe, and not infrequently, catastrophic or life threatening adverse events. Rituximab is a B-cell depleting agent that has been used in treating pemphigus patients that are non-responsive to conventional therapy, or have developed side effects to it or have contraindications to their use.

Areas covered: This expert opinion based on a thorough review of the literature focuses on the use of rituximab in the treatment of pemphigus. The main areas of coverage include its market needs, clinical outcomes, adverse events and prospective competitors in the future.

Expert opinion: Rituximab is an effective agent in the treatment of pemphigus. One of the unique features of rituximab is that it produces rapid control of clinical disease and cessation of new blisters formation. There is no consensus on the most effective protocol. It has been shown that the addition of intravenous immunoglobulin can provide long lasting remission. Rituximab may alter the regulation of the immune system. Physicians should be aware of its potential side effects.

KEYWORDS:

• Autoimmune diseases
• blistering diseases
• biologic agents
• B-cell depletion
• immune regulation
• intravenous immunoglobulin
• pemphigus vulgaris

Box 1. Drug summary

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.

Additional information

Funding

This study was supported in part by an unrestricted educational, grant from the Dysimmune Disease Foundation (USA).