Egan C A, Meadows K P, Zone J J
Medicine Service, Section of Dermatology, Salt Lake City Veterans Affairs Medical Center and Department of Dermatology, University of Utah School of Medicine, Salt Lake City, Utah, USA.
Int J Dermatol. 2000 Mar;39(3):230-5. doi: 10.1046/j.1365-4362.2000.00876.x.
Bullous pemphigoid is an immunobullous disease affecting predominantly older patients. In severe cases, high-dose corticosteroids and/or other immunosuppressants are often needed long term to control the disease. These can be associated with serious side-effects in this patient population. Objective To evaluate the benefit of plasmapheresis as a steroid saving agent in a cohort of 10 patients.
Plasmapheresis was effective as a steroid saving therapy. All patients went into remission with a lower daily dosage of oral prednisone at 3 and 6 months postplasmapheresis. Two patients had side-effects from therapy that, while significant, did not interfere with long-term improvement in their disease. Eight patients had circulating immunoglobulin G (IgG) antibodies reactive with bullous pemphigoid antigen 1, and three of these had circulating antibodies reactive with bullous pemphigoid antigen 2 on Western immunoblot.
Plasmapheresis was an effective steroid sparing therapy in these patients. Due to its high cost and potential morbidity, plasmapheresis should not be recommended as routine therapy for bullous pemphigoid, but it is a useful adjunct in resistant cases.
大疱性类天疱疮是一种主要影响老年患者的免疫性疱病。在严重病例中,常需要长期使用高剂量皮质类固醇和/或其他免疫抑制剂来控制疾病。这些药物在该患者群体中可能会带来严重的副作用。目的:评估血浆置换作为类固醇节省剂在10例患者队列中的益处。
血浆置换作为类固醇节省疗法有效。所有患者在血浆置换后3个月和6个月时,口服泼尼松的日剂量降低,病情均得到缓解。两名患者出现治疗副作用,虽然严重,但并未干扰其疾病的长期改善。8例患者循环免疫球蛋白G(IgG)抗体与大疱性类天疱疮抗原1反应,其中3例在免疫印迹法检测中循环抗体与大疱性类天疱疮抗原2反应。
血浆置换对这些患者是一种有效的类固醇节省疗法。由于其成本高和潜在的发病率,血浆置换不应被推荐为大疱性类天疱疮的常规治疗方法,但在难治性病例中是一种有用的辅助治疗方法。