Sami Naveed
University of Alabama at Birmingham, 1520 3rd Ave., South, EFH 414, Birmingham, AL 35294-0009, USA.
Ear Nose Throat J. 2017 Apr-May;96(4-5):E35-E38.
Pemphigus vulgaris is a potentially fatal autoimmune blistering disease that can involve the nasopharyngeal and laryngeal tissues. The disease can be recalcitrant to conventional oral treatments, and treatment alternatives are limited. This retrospective study evaluated the efficacy of rituximab as a rescue agent in 5 patients with recalcitrant pemphigus vulgaris involving nasopharyngeal and laryngeal mucosa. All 5 patients were unresponsive to systemic steroids and at least one conventional oral immunosuppressive agent. The patients received rituximab infusions as a rescue agent because of recalcitrant disease. All 5 patients had a complete clinical response to rituximab and could discontinue systemic steroids and reduce the dosage of their initial immunosuppressive agent. No major adverse reactions were observed or reported with rituximab. Rituximab can be used as an effective rescue agent in the treatment of severe pemphigus vulgaris with nasopharyngeal and laryngeal involvement.
寻常型天疱疮是一种可能致命的自身免疫性水疱病，可累及鼻咽和喉部组织。该疾病对传统口服治疗可能具有顽固性，且治疗选择有限。这项回顾性研究评估了利妥昔单抗作为挽救药物治疗5例累及鼻咽和喉黏膜的顽固性寻常型天疱疮患者的疗效。所有5例患者对全身用类固醇和至少一种传统口服免疫抑制剂均无反应。由于疾病顽固，这些患者接受利妥昔单抗输注作为挽救药物。所有5例患者对利妥昔单抗均有完全的临床反应，且可停用全身用类固醇并减少初始免疫抑制剂的剂量。未观察到或报道利妥昔单抗有重大不良反应。利妥昔单抗可作为治疗累及鼻咽和喉部的重度寻常型天疱疮的有效挽救药物。
