接受免疫吸附或利妥昔单抗辅助治疗的天疱疮患者的临床及免疫学随访
Clinical and immunological follow-up of pemphigus patients on adjuvant treatment with immunoadsorption or rituximab.
作者信息
Pfütze Martin, Eming Rüdiger, Kneisel Andrea, Kuhlmann Uwe, Hoyer Joachim, Hertl Michael
机构信息
Department of Dermatology and Allergology, Philipps University, Marburg, Germany.
出版信息
Dermatology. 2009;218(3):237-45. doi: 10.1159/000187431. Epub 2008 Dec 16.
BACKGROUND
Pemphigus vulgaris (PV) is a life-threatening autoimmune blistering skin disease which is associated with pathogenic IgG autoantibodies against desmogleins (Dsg) 1 and 3. Novel therapeutic strategies such as immunoadsorption (IA) or the anti-CD20 antibody rituximab (Rtx) hold promise to be effective in severe or recalcitrant PV.
PATIENTS AND METHODS
In the present retrospective study, 6 patients with extensive cutaneous PV were subjected to adjuvant IA treatment while 5 patients with severe mucosal PV received adjuvant Rtx treatment.
RESULTS
Within 6 months, IA and Rtx induced excellent clinical responses which were associated with a significant reduction of prednisolone doses and a decrease in anti-Dsg-specific IgG. Over a 12-month period, 3 IA-treated patients required additional adjuvant drugs while all of the PV patients on Rtx had no or only minimal residual symptoms.
CONCLUSION
The relative therapeutic (long-term) efficacy of IA and Rtx in cutaneous versus mucosal PV needs to be evaluated in a prospective study.
背景
寻常型天疱疮（PV）是一种危及生命的自身免疫性水疱性皮肤病，与抗桥粒芯糖蛋白（Dsg）1和3的致病性IgG自身抗体有关。免疫吸附（IA）或抗CD20抗体利妥昔单抗（Rtx）等新型治疗策略有望有效治疗重度或难治性PV。
患者与方法
在本回顾性研究中，6例广泛皮肤型PV患者接受辅助IA治疗，5例重度黏膜型PV患者接受辅助Rtx治疗。
结果
6个月内，IA和Rtx均诱导出良好的临床反应，这与泼尼松龙剂量显著减少及抗Dsg特异性IgG降低有关。在12个月期间，3例接受IA治疗的患者需要额外的辅助药物，而所有接受Rtx治疗的PV患者均无或仅有轻微残留症状。
结论
IA和Rtx在皮肤型与黏膜型PV中的相对治疗（长期）疗效需要在前瞻性研究中进行评估。
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