Objectives: This pilot retrospective study examined the role of continuous low-dose maintenance immunomodulatory treatment (IMT) as an adjunct to rituximab (RTX) rescue therapy in severe pemphigus vulgaris (PV) and pemphigus foliaceus (PF) after a complete response (CR) to RTX was achieved. Materials and methods: Ten severe pemphigus patients who received RTX rescue therapy were evaluated after achieving CR. The post-RTX clinical course and long-term follow up was compared between patients who adhered to a low recommended dose (LRMD) to patients who were non-compliant with LRMD. Results: Five patients relapsed due to discontinuing or tapering their LRMD therapy, whereas the five patients who adhered to their maintenance therapy did not experience a relapse after the initial post-RTX CR. A combination of increasing or adding IMTs and initiating subsequent RTX cycles was used to regain control in relapsed patients. Conclusions: We propose an alternative treatment strategy utilizing RTX as a rescue agent in combination with long-term LRMD as a means to maintain a sustained long-term CR post-RTX therapy in severe pemphigus patients. This strategy could prevent disease flares and the need for additional RTX cycles and higher dosages of immunomodulatory therapies.
Keywords: Immunomodulatory therapy; pemphigus foliaceus; pemphigus vulgaris; rituximab.