Researchers in France examined and said that Rituximab can be administered alone, without systemic corticosteroids, to prevent relapse in patients with severe pemphigus. The study has been published online in JAMA Dermatology.

All had been treated with at least one cycle of rituximab for steroid dependence, resistance or adverse events. “Pemphigus is a rare, chronic, relapsing, and potentially life-threatening autoimmune bullous dermatosis,” state Dr. Philippe Bernard of Reims University Hospital and colleagues. “European guidelines recommend administration of systemic corticosteroids as a first-line therapy.”

To assess whether rituximab could be used instead of steroids to maintain remission, the team conducted a single-center, retrospective case-series study of adults with a confirmed diagnosis of pemphigus vulgaris or foliaceus. Of 27 patients who received at least one cycle of rituximab, 11 (median age, 42; five women) received additional cycles of the drug as maintenance therapy without any other treatment.

Maintenance monotherapy consisted of a single rituximab infusion (1 g) given every six months, in some cases decreasing after 18 months to once yearly. The median duration of treatment was 33 months, and the median number of infusions was six. All 11 patients remained in complete remission during maintenance therapy, with no adverse events.

However, five of the 11 experienced grade 3 or 4 adverse events between the initial cycle and the first rituximab maintenance infusion: one had sepsis; one had hypertension; and two each had diabetes or endocrine disorders. The 11 patients were in complete remission at last follow-up (median, 78 months after the first rituximab cycle), with 10 having discontinued rituximab.

Dr. Adam Friedman, Director of Translational Research and of the Supportive Oncodermatology Clinic at George Washington School of Medicine and Health Sciences in Washington, DC, commented, “Pemphigus vulgaris is fortunately a rare but sadly chronic, life-altering and even life-threatening autoimmune blistering disease for which we have few effective treatments.

“The age of biologic therapies has changed the landscape for the treatment of inflammatory skin diseases and even skin cancer,” he explained in an email to Reuters Health. “Rituximab is not approved for pemphigus but is well known for its efficacy in treating this disabling disease.”

“I applaud these investigators for taking the creative initiative to evaluate long-term maintenance,” he said. “We always have to think about the long game even once the condition is under control.” “It is disappointing that in this age, insurance dictates a patient's treatment, not the physician,” he observed. “We must, however, push on and continue to encourage studies such as these to reveal potential treatment algorithms that can be effective and safe long-term.”

Authors conclude the results of this case series indicated that rituximab can be used as single maintenance therapy, without a systemic corticosteroid, with good efficacy and tolerance in patients having severe pemphigus requiring long-term therapy for prevention.