Which drugs are used in the treatment of pemphigus vulgaris?

Updated: Jun 14, 2018
  • Author: Bassam Zeina, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
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Answer

Corticosteroids have improved overall mortality, but now much of the mortality and morbidity in these patients relates to the adverse effects of therapy. Whether massive doses of steroids have any advantage over doses of 1 mg/kg/d is unclear. [39]

Immunosuppressive drugs are steroid sparing and should be considered early in the course of the disease. Epidermal growth factor may speed healing of localized lesions. [40]  Many authorities now use rituximab, the anti-CD20 antibody, as first- or second-line therapy, with evidence from 2017 suggesting better outcomes when rituximab is used as a first-line agent. [41, 42, 43, 44, 45, 46, 47, 48, 49]  

Rituximab was approved by the US Food and Drug Administration (FDA) for treatment of pemphigus vulgaris in June 2018. Approval was based on a randomized trial that compared rituximab plus oral corticosteroid treatment with corticosteroid treatment alone as first-line therapy for patients with moderate-to-severe pemphigus vulgaris. [49] At month 24, 41 (89%) of 46 patients assigned to rituximab plus short-term prednisone were in complete remission off-therapy compared with 15 (34%) of 44 assigned to prednisone alone (P<.0001).

The antitumor necrosis factor drugs sulfasalazine and pentoxifylline have been reported as effective adjunctive treatments, reducing the serum level of tumor necrosis factor and resulting in rapid clinical improvement. [50] Methotrexate has also been used. [51, 52] Dapsone has been suggested as a steroid-sparing agent in the maintenance phase of pemphigus vulgaris treatment [53, 54] ; dapsone has also been suggested as a first-line agent. [55]

Intravenous immunoglobulin therapy has been suggested as efficacious in pemphigus vulgaris treatment. [56, 57, 58, 59] Amagai et al reported on the successful use of intravenous immunoglobulin in pemphigus patients who did not fully respond to systemic steroids, [60] and Asarch et al reported its use in pediatric patients. [61]

Infliximab has proven effective in some patients with refractory disease, and photodynamic therapy has been suggested as a possible adjunctive treatment for recalcitrant ulceration. [62] Mizoribine has been used for refractory ocular manifestations. [63]  

Plasmapheresis has been used in refractory cases, usually in conjunction with cytotoxic therapy. [64]


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