The evolving role of rituximab in the treatment of pemphigus vulgaris: a comprehensive state-of-the-art review

ABSTRACT

Introduction

Pemphigus vulgaris (PV) is a life-threatening autoimmune mucocutaneous blistering disease. Systemic corticosteroids (CS), while life-saving, have several serious side effects. To improve treatment and prognosis, recently rituximab (RTX), a chimeric monoclonal antibody against CD20 molecule on B cells, has become popular. This Expert Opinion discusses clinical and scientifically relevant aspects of RTX treating PV.

Area Covered

This presentation describes the mechanism of action, clinical efficacy, safety, adverse events, protocols used, and clinical outcomes. Concerns for infection, reactivation of latent or previous infections, and high relapse rate are discussed.

Expert opinion

Use of RTX in PV is still a work in progress. There are many unanswered questions. FDA did not provide a protocol or guidelines. Whenever RTX is used, systemic corticosteroids are simultaneously used, albeit for a shorter duration and lower dose. Used in these doses for these durations they can cause immunosuppression. Would it be more appropriate if instead of ‘First Line Therapy’ it would be more advisable to use the term ‘First Adjunctive Immunosuppressive Agent’?

KEYWORDS:

• Pemphigus vulgaris
• rituximab
• relapse rates
• b cell depletion therapy
• adverse events
• long-term remissions
• combination with intravenous immunoglobulin
• remission rates
• desmoglein autoantibodies

Acknowledgments

The authors are grateful to Merve Aksoy MD and Mughees Farooq MD for their help in the preparation of this manuscript.

Declaration of interest

The authors have no other 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 relationships or otherwise to disclose.

Additional information

Funding

This study was supported in part, by an unrestricted educational grant from the Dysimmune Diseases Foundation, Boston, MA, USA.