Dietze Jenny, Hohenstein Bernd, Tselmin Sergey, Julius Ulrich, Bornstein Stefan R, Beissert Stefan, Günther Claudia
Department of Dermatology, University Hospital and Carl Gustav Carus Faculty of Medicine, Technische Universität Dresden, Dresden, Germany.
Extracorporeal Treatment and Apheresis Center, Department of Medicine III, University Hospital and Carl Gustav Carus Faculty of Medicine, Technische Universität Dresden, Dresden, Germany.
Atheroscler Suppl. 2017 Nov;30:271-277. doi: 10.1016/j.atherosclerosissup.2017.05.044. Epub 2017 Jun 3.
Pemphigus vulgaris is a chronic autoimmune disease characterized by blisters and erosions forming in the mucous membranes and the skin. Many patients are severely impaired by pain, weight loss and increased risk of infections. The disease is mediated by specific autoantibodies directed against desmogleins that contribute to connect keratinocytes in the epidermis. Autoantibody deposition in the skin causes inflammation and intraepidermal akantholysis. The concentration of autoantibodies in serum correlates with disease activity. Therefore, the removal of autoantibodies by immunoadsorption is a targeted therapeutic intervention for patients with pemphigus vulgaris.
A total of 9 patients with pemphigus vulgaris resistant to the standard treatment regimen were treated by immunoadsorption using the TheraSorb™-Ig adsorber system and analyzed retrospectively. Patients received immunoadsorption on two or four consecutive days. Cycles were repeated every two or four weeks, respectively. Treatment was performed for a mean period of 17.5 months (range 6-26). Outcome was measured as improvement in clinical disease analyzed by the investigators global assessment and the reduction of autoantibodies in serum measured by indirect immunofluorescence and ELISA. Tolerability of treatment by patients was evaluated using a visual analog scale.
Retrospective analysis of 9 patients consecutively treated by immunoadsorption revealed an 80% reduction of the autoantibody concentration in serum after 6 months of treatment, led to a clinical improvement of disease in combination with classical immunosuppression. Steroid consumption could be reduced by 50% after 30 and 75% after 90 days. Therapy resulted in a total response rate of 89%, with 56% of patients reaching partial and 33% complete remission. The bi-weekly treatment regimen resulted in effective improvement of disease and was in favor to the 4-weekly regimen by the subjective judgment of tolerability by the patients.
Immunoadsorption for the treatment of pemphigus vulgaris is safe and effective. The good tolerability of a bi-weekly treatment regimen shown here might be a valuable therapeutic option in further studies defining the optimal frequency of immunoadsorption required in treatment of pemphigus.
寻常型天疱疮是一种慢性自身免疫性疾病，其特征为在黏膜和皮肤形成水疱及糜烂。许多患者因疼痛、体重减轻和感染风险增加而严重受损。该疾病由针对桥粒芯糖蛋白的特异性自身抗体介导，这些抗体在表皮中参与连接角质形成细胞。皮肤中的自身抗体沉积会引发炎症和表皮内棘层松解。血清中自身抗体的浓度与疾病活动度相关。因此，通过免疫吸附去除自身抗体是寻常型天疱疮患者的一种靶向治疗干预措施。
对9例对标准治疗方案耐药的寻常型天疱疮患者采用TheraSorb™-Ig吸附系统进行免疫吸附治疗，并进行回顾性分析。患者连续两天或四天接受免疫吸附治疗。周期分别每两周或四周重复一次。治疗平均进行17.5个月（范围6 - 26个月）。通过研究者整体评估分析临床疾病的改善情况，并通过间接免疫荧光和酶联免疫吸附测定法测量血清中自身抗体的减少情况来衡量治疗结果。使用视觉模拟量表评估患者对治疗的耐受性。
对9例连续接受免疫吸附治疗的患者进行回顾性分析发现，治疗6个月后血清中自身抗体浓度降低了80%，与传统免疫抑制联合使用可使疾病得到临床改善。30天后类固醇用量可减少50%，90天后减少75%。治疗的总有效率为89%，其中56%的患者达到部分缓解，33%的患者达到完全缓解。两周一次的治疗方案使疾病得到有效改善，并且根据患者对耐受性的主观判断，该方案优于四周一次的治疗方案。
免疫吸附治疗寻常型天疱疮安全有效。此处显示的两周一次治疗方案的良好耐受性可能是进一步研究确定寻常型天疱疮治疗所需免疫吸附最佳频率时的一种有价值的治疗选择。
