A Systematic Review of Treatment Options and Clinical Outcomes in Pemphigoid Gestationis.

A Systematic Review of Treatment Options and Clinical Outcomes in Pemphigoid Gestationis.
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类天疱疮妊娠的治疗方案和临床结果的系统综述。

DOI:
10.3389/fmed.2020.604945
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发表时间:
2020
影响因子:
3.9
通讯作者:
Marzano AV
Marzano AV
中科院分区:
医学3区
文献类型:
--
作者:
Genovese G;Derlino F;Cerri A;Moltrasio C;Muratori S;Berti E;Marzano AV

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背景:类天疱疮妊娠(PG)的治疗方案是不标准化的,大多数证据来自个案报告或小系列。目的:系统地回顾目前关于PG的治疗和临床结果的文献,并对其治疗管理提出建议。方法:根据PRISMA指南设计先验方案。在PubMed、Scopus和Web of Science数据库中检索了1970年至2020年3月期间发表的详细介绍PG治疗和临床结果的英文文章。结果:共分析109篇文献,其中PG患者140例。没有发现详细描述PG治疗的随机对照试验或可靠的观察性研究。全身性糖皮质激素±局部糖皮质激素和/或抗组胺药是最常用的治疗方式(n = 74/137; 54%)。136例患者中有114例(83.8%)达到完全缓解。64例(45.7%)患者因副作用或无效接受了一种以上的治疗方式。在轻度疾病患者中,暂且不考虑局部皮质类固醇作为单药治疗±抗组胺药,全体皮质类固醇±局部皮质类固醇和/或抗组胺药可使比例最高的患者(83%)完全缓解,而保留类固醇治疗±局部皮质类固醇和/或抗组胺药与最低比例的发作相关(55.5%)。局限性:本综述是根据数量有限的单个病例报告和小病例系列撰写的。轻至中度PG患者的漏报/漏诊、部分/缺失随访、缺乏对新生儿结局的精确描述以及缺乏有效的测量疾病严重程度的客观评分是本研究的其他局限性。我们的系统综述受到发表偏倚的影响。结论:全身性皮质类固醇是PG最常用的治疗方法。虽然大多数患者完全缓解,但其中许多患者有难治性/持续性疾病,需要多线治疗。因此,我们提供了一种PG治疗的算法,将本系统综述的结果与目前可用于大疱性类天疱疮的知识相结合。高质量的研究将进一步帮助评估不同治疗方案对PG的有效性。
Background: Treatment regimens for pemphigoid gestationis (PG) are non-standardized, with most evidence derived from individual case reports or small series. Objectives: To systematically review current literature on treatments and clinical outcomes of PG and to establish recommendations on its therapeutic management. Methods: An a priori protocol was designed based on PRISMA guidelines. PubMed, Scopus, and Web of Science databases were searched for English-language articles detailing PG treatments and clinical outcomes, published between 1970 and March 2020. Results: In total, 109 articles including 140 PG patients were analyzed. No randomized controlled trials or robust observational studies detailing PG treatment were found. Systemic corticosteroids ± topical corticosteroids and/or antihistamines were the most frequently prescribed treatment modality (n = 74/137; 54%). Complete remission was achieved by 114/136 (83.8%) patients. Sixty-four patients (45.7%) were given more than one treatment modality due to side effects or ineffectiveness. Leaving aside topical corticosteroids as monotherapy ± antihistamines in patients with mild disease, systemic corticosteroids ± topical corticosteroids and/or antihistamines led to complete remission in the highest proportion of patients (83%), while steroid-sparing treatments ± topical corticosteroids and/or antihistamines were associated with the lowest proportion of flares (55.5%). Limitations: The review has been drafted based on a limited number of single case reports and small case series. Underreporting/underdiagnosis of patients with mild-to-moderate PG, partial/absent follow-up, absence of precise description of neonatal outcomes and lack of validated objective scores for measuring disease severity are other limitations of our study. Our systematic review was affected by publication bias. Conclusion: Systemic corticosteroids are the most frequently used treatment for PG. Whilst most patients achieve complete remission, many of them have refractory/persistent disease requiring multiple lines of therapy. Therefore, we provided an algorithm for PG treatment integrating the results of this systematic review with current knowledge available for bullous pemphigoid. High-quality studies will further help assess the effectiveness of different treatment options for PG.
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