Rituximab therapy in pemphigus: A long-term follow-up

Abstract

group after a mean period of 18.2 months (p = .0588), managed with systemic immunosuppressants or rituximab re-infusion (500 mg).Our study confirms the efficacy of rituximab in patients with a difficult-to-treat pemphigus: 96.6% of our patients experienced partial or complete remission, similarly to the literature data. However, a complete clinical remission rate, documented by declining levels of anti-Dsg 1 and anti-Dsg 3, was lower than in other reported series (48.3% vs. 65-78%;Gregoriou, 2015;Wang, 2015).The debate regarding which protocol is optimal is still open. Apparently the rheumatologic protocol is the most appropriate, as pemphigus is an autoimmune disease in which B-cell clone eradication is not necessary and unsafe. Our study is the first to document a significantly greater efficacy of the oncologic protocol: despite similar remission rates among the two groups, the oncologic protocol showed better results in terms of complete clinical remission and shorter relapse-free score, without enhanced adverse effects. Remission depends on number of parameters. In our series, no significant differences were observed among disease severity. Actually, the patients treated with the oncologic protocol presented unfavorable prognostic factors, as more past treatments (mean 5.4 vs. 2.5, p < .0001) and longer disease duration (mean 60.8 vs. 39.7 months, nonstatistically significant).Our preliminary data, although needing to be confirmed by further studies, suggest that oncologic dosing protocol may be preferred to rheumatologic protocol in pemphigus patients requiring a secondline therapy.

Long‐term outcomes of rituximab therapy in pemphigus

“…Three other reports provide median follow-up times of longer than three years after rituximab treatment. 7,14,15 However, in all these studies a substantial share of relapsing patients was not given repeat rituximab or the outcomes of renewed rituximab therapy were not clearly stated by the authors or both. 7,14,15 The relapse rate in our patients declined with the increasing number of rituximab cycles.…”

“…7,14,15 However, in all these studies a substantial share of relapsing patients was not given repeat rituximab or the outcomes of renewed rituximab therapy were not clearly stated by the authors or both. 7,14,15 The relapse rate in our patients declined with the increasing number of rituximab cycles. This phenomenon has already been observed in other studies on pemphigus 9,10,14 and is also known to occur in rheumatoid arthritis.…”

“…Some patients recurred as late as 156 months after a successful rituximab therapy, underscoring the importance of long‐term follow‐up for reliable estimates of final treatment outcomes. Three other reports provide median follow‐up times of longer than three years after rituximab treatment 7,14,15 . However, in all these studies a substantial share of relapsing patients was not given repeat rituximab or the outcomes of renewed rituximab therapy were not clearly stated by the authors or both 7,14,15 .…”

Case of a severe vulgaris and foliaceus pemphigus in a young patient treated with rituximab, with subsequent development of chronic urticaria

“…Due to the severity of the clinic (Figure 2), on January 2018, the patient underwent therapy with rituximab (RTX), a monoclonal antibody targeting CD20 and increasingly used for its effectiveness on pemphigus. 5 Two different dosing protocols of RTX are used in the literature 6 : a traditional oncologic protocol (4 weekly infusions, 375 mg/ m 2 of body surface) and a more recent rheumatoid arthritis protocol (a single 1000 mg infusion repeated after 15 days); we chose the latter. This therapy allows a fast and complete healing from pemphigus in just a few weeks (Figure 3), with autoantibody negativization.…”

Therapeutic approaches and targets for treatment of autoimmune bullous diseases

“…The latter group of patients can benefit from additional cycles of rituximab; this suggests that a maintenance therapy can be administered, even if the exact timing of retreatment is not clearly defined 46 . Maintenance therapy may consist of 500 mg at month 12 and every 6 months thereafter or based on clinical assessment 50 . According to Lunardon et al, rituximab should be started early during the disease course in order to achieve better outcomes 51…”