A.J. RobinsonM.Vu, G.A. Unglik, G. Varigos. Scardamaglia
Pemphigus vulgaris is a chronic, autoimmune B-cell medi-ated blistering disease, associated with significant morbid-ity and mortality. Rituximab has proven effective fortreatment of steroid-refractory pemphigus in multiple stud-ies, although there is controversy regarding the optimumdose for inducing remission. Additionally, there is a pau-city of published evidence regarding the optimal adjuvanttherapy for managing this difficult condition, and effectivedisease control often requires long-term immunosuppres-sion, even in disease-free periods. We present a case seriesof single-centre long-term follow up of twelve patientswith pemphigus, treated with Rituximab along with con-current adjuvant therapy. Nine patients were treated withtwo 500 mg doses of Rituximab separated by 14 days, anda further three earlier patients were treated with doses of375/m2, receiving either two, three or four infusions intotal. In all patients, Rituximab resulted in B-cell depletion,along with a reduction in blistering disease. Three of thepatients treated with low-dose Rituximab required repeatdosing cycles, due to either relapsed disease or incomplete disease control following the first dosing cycle, and haveremained disease free out to as long as 146 weeks thusfar. Low-dose Rituximab, with concurrent use of mycophe-nolate mofetil (MMF) appeared to be a safe and effectivemeans of inducing and maintaining remission in our caseseries. Based on our experience and a review of the litera-ture, we propose a protocol for the use of Rituximab andconcurrent adjuvant therapy with MMF for the treatmentof pemphigus.