Rituximab in the Treatment of Pemphigus Vulgaris
- Labib R. ZakkaAffiliated withCenter for Blistering Diseases
- , Shawn S. ShettyAffiliated withCenter for Blistering Diseases
- , A. Razzaque AhmedAffiliated withCenter for Blistering Diseases Email author
Rituximab is increasingly used in patients with pemphigus vulgaris (PV) who are nonresponders to conventional therapy.
A PubMed search was conducted using the words pemphigus vulgaris and rituximab therapy from papers published between 2000 and 2012. Two protocols were used. In the lymphoma protocol, patients received four weekly infusions of rituximab (dose 375 mg/m2). The rheumatoid arthritis (RA) protocol consisted of two infusions of 1,000 mg each 15 days apart. The variables recorded from each study included clinical remission off or on therapy, relapse rate, incidence of serious adverse events, concomitant therapies, duration of follow-up, and when available, levels of B cells and autoantibodies.
Forty-two studies were found, which reported 272 patients; 180 were treated by the lymphoma protocol and 92 by the RA protocol. Both protocols were effective in treating recalcitrant PV. The lymphoma protocol had a lower response rate, relapse rate and serious infections, but higher mortality, and there were nonresponders. The RA protocol produced a higher response rate, relapse rate, number of infections, but lower mortality rate, and lacked nonresponders. The cumulative follow-up for patients treated with the lymphoma protocol was 15.44 months (range 1–41) and 21.04 months (range 8.35–29) for the RA protocol. A major concern in both protocols was the high infection rates, some of which were fatal. A different protocol using a combination of rituximab with intravenous immunoglobulin in a defined manner with a definitive endpoint, used in a limited cohort of patients, showed promising results.
Neither protocol produced a sustained clinical remission and both required continued systemic therapy. Before initiation of treatment, physicians should have a specific goal and endpoint and be aware of its potential side effects and lack of information on its long-term effects. Patients should be carefully monitored during and after therapy.
KeywordsClinical outcomes Immunology and inflammatory skin diseases Lymphoma protocol Pemphigus vulgaris Rheumatoid arthritis protocol Rituximab
- Rituximab in the Treatment of Pemphigus Vulgaris
- Open Access
- Available under Open Access This content is freely available online to anyone, anywhere at any time.
Dermatology and Therapy
- Online Date
- November 2012
- Print ISSN
- Online ISSN
- Springer Healthcare Communications
- Additional Links
- Clinical outcomes
- Immunology and inflammatory skin diseases
- Lymphoma protocol
- Pemphigus vulgaris
- Rheumatoid arthritis protocol