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Pemphigus vulgaris (PV) is an autoimmune blistering disease affecting the skin and/or mucosa. Rituximab (RTX) has been approved recently by US FDA as an effective and safe treatment of PV. The high incidence of PV in Iran encouraged our team to prepare a consensus guideline for RTX administration based on literature review and a decade experience of an expert panel. RTX is recommended for the treatment of new cases of PV as well as patients not responding to conventional therapy. Contraindications include history of anaphylaxis or IgE-mediated hypersensitivity to murine proteins of RTX, severe active infections, pregnancy, breastfeeding, severe heart failure, and arrhythmia. Prophylactic antiviral therapy is recommended in patients at risk of reactivation of HBV and isoniazid for those at risk of reactivation of tuberculosis. Concomitant use of systemic corticosteroids is recommended as a rule. Except for methotrexate, the combination with other immunosuppressive drugs is discouraged. Intravenous immunoglobulin is recommended for those at risk of infections or with extensive disease. The recommended dosage of RTX for the first cycle is 2 g either 500 mg weekly or 1 g biweekly. There is no general consensus whether the next doses of RTX be administered upon relapse or as maintenance therapy. We strongly recommend RTX sooner in the course of pemphigus., (© 2019 Wiley Periodicals, Inc.)

During the last decade, successfully treatment of patients diagnosed with pemphigus vulgaris (PV) with rituximab (RTX) was reported by several authors. The present study has been designed to compare the clinical outcomes and RTX-related side effects between the two groups of early treated (≤ 6 months) and lately treated PV (> 6 months) patients with RTX. We did a retrospective study between Oct 2014 and Jun 2016 to compare the short-term efficacy and safety of RTX in PV diagnosed patients. The primary and secondary endpoints were complete/partial remission of disease and safely tapering of corticosteroids without disease relapse, respectively. Among the 250 RTX exposed PV patients in the selected period, 107 were successfully followed for the mean 19.71 ± 16.78 months. Twenty-four and eighty three have categorized as the early (≤ 6 months after diagnosis) or lately (> 6 months after diagnosis) RTX-treated patients, respectively. A higher rate of complete remission, longer time lasting remission phase, and a lower number of adjuvants were associated with early RTX treatment. Early treatment with RTX might be associated with improvement of clinical effects, but does not seems to be safer than lately RTX therapy. Those in the early treated group may not only have a higher chance to achieve complete remission, but also experience a longer time of disease remission with lower cumulative doses of adjuvant therapy.