Patterns of remission in pemphigus vulgaris
Patterns of remission in pemphigus vulgaris
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DOI：
10.1016/s0190-9622(00)90213-5
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发表时间：
2000-03-01
影响因子：
13.8
通讯作者：
Bystryn, JC
中科院分区：
文献类型：
--
作者：
Herbst, A;Bystryn, JC
背景：天疱疮缓解的发生率尚不清楚，因为这些缓解通常在疾病进展的单个点报告。因此，它是不确定的治疗是否简单地抑制了疾病的表现，因此必须继续管理，或诱导完全和持久的缓解，允许治疗中断。目的：为了回答这个问题，我们调查了缓解的发生率在一个长期的纵向研究方法：诱导完全和持久的缓解（至少6个月内无病变且未接受全身治疗）结果：40例寻常型天疱疮患者中有5例（5%）死于该病，平均随访7.7年。在诊断后2年、5年和10年，分别有25%、50%和75%的患者诱导了完全缓解和长期缓解。其余大部分患者部分缓解或有轻微的疾病控制与小剂量的类固醇。疾病的过程遵循不同的模式，一些患者迅速进入完全和持久的缓解，而另一些患者从未进入完全缓解。诱导完全缓解与疾病的初始严重程度和程度以及对treatment.Conclusion的耳反应有关：有可能最终诱导大部分天疱疮患者的完全和持久缓解，使全身治疗安全地停止，而不会在疾病活动中出现耀斑。能够实现这一目标的患者比例随着时间的推移而稳步增加，大约75%的患者在10年后可以停止治疗。
Background: The incidence of remissions in pemphigus is unclear because these are usually reported at a single point in the evolution of the disease. Thus it is uncertain whether treatment simply suppresses the manifestations of the disease and consequently must be continuously administered, or induces complete and long-lasting remissions that permit therapy to be discontinued.Objective: To answer this question, we investigated the incidence of remission in a long-term longitudinal studyMethods: The induction of complete and long-lasting remissions (lesion free with no systemic therapy for at least 6 months) was studied in 40 patients with pemphigus vulgaris treated conventionally and followed up for an average of 7.7 years by the same investigator.Results: Five (5%) of the patients died of the disease. Complete and long-lasting remissions were induced in 25%, 50%, and 75% of patients 2, 5, and 10 years, respectively, after diagnosis. Most of the remaining patients were in partial remission or had mild disease controlled with a small dose of steroids. The course of the disease followed different patterns, with some patients rapidly entering complete and long-lasting remissions, whereas others never entered into a complete remission. The induction of complete remission was related to the initial severity and extent of disease and to ear ly response to treatment.Conclusion: It is possible to eventually induce complete and durable remissions in most patients with pemphigus that permit systemic therapy to be safely discontinued without a flare in disease activity. The proportion of patients in whom this can be achieved increases steadily with time, and therapy can be discontinued in approximately 75% of patients after 10 years.