当前位置: X-MOL 学术J. Allergy Clin. Immunol. › 论文详情
Our official English website, www.x-mol.net, welcomes your feedback! (Note: you will need to create a separate account there.)
Pediatric recurrent fever and autoinflammation from the perspective of an allergist/immunologist
Journal of Allergy and Clinical Immunology ( IF 14.2 ) Pub Date : 2020-09-28 , DOI: 10.1016/j.jaci.2020.09.019
Lori Broderick 1 , Hal M Hoffman 1
Affiliation  

Autoinflammatory diseases are monogenic and polygenic disorders due to dysregulation of the innate immune system. The inherited conditions have been clustered with primary immunodeficiencies in the latest practice parameters; however, these diseases have unique clinical presentations, genetics, and available therapies. Given the presentation of fevers, rashes, and mucosal symptoms observed in many of these syndromes, patients are likely to present to an allergist/immunologist. Although there has been attention in the literature to diagnosis and treatment of rare, genetically defined autoinflammatory disorders, physicians are challenged by increasing numbers of patients with intermittent or periodic fevers who face unnecessary morbidities due to a lack of a diagnosis. The broad differential of diseases presenting with fever includes autoinflammatory syndromes, infections associated with immunodeficiency and/or allergies complicated by infection, and less commonly, autoimmune disorders or malignancy. To address this challenge, we review the history of the medical approach to fever, current diagnostic paradigms, and controversies in management. We describe the spectrum of disorders referred to a recurrent fever disorders clinic established in an Allergy/Immunology division at a tertiary pediatric care center. Finally, we provide practical recommendations including historical features and initial laboratory investigations that can help clinicians appropriately manage these patients.



中文翻译:

从过敏症专家/免疫学家的角度看小儿反复发热和自身炎症

自身炎症性疾病是由于先天免疫系统失调引起的单基因和多基因疾病。在最新的实践参数中,遗传疾病与原发性免疫缺陷聚集在一起;然而,这些疾病具有独特的临床表现、遗传学和可用的治疗方法。鉴于在许多这些综合征中观察到的发热、皮疹和粘膜症状,患者很可能就诊于过敏症专科医生/免疫学家。尽管文献中一直关注罕见的、遗传定义的自身炎症性疾病的诊断和治疗,但越来越多的间歇性或周期性发热患者由于缺乏诊断而面临不必要的并发症,这给医生带来了挑战。发热性疾病的广泛差异包括自身炎症综合征、与免疫缺陷相关的感染和/或过敏症并发感染,以及较少见的自身免疫性疾病或恶性肿瘤。为了应对这一挑战,我们回顾了发烧医学方法的历史、当前的诊断范式和管理中的争议。我们描述了在三级儿科护理中心过敏/免疫科设立的复发性发热疾病诊所所涉及的疾病谱。最后,我们提供实用的建议,包括历史特征和初步实验室调查,可以帮助临床医生适当地管理这些患者。自身免疫性疾病或恶性肿瘤。为了应对这一挑战,我们回顾了发烧医学方法的历史、当前的诊断范式和管理中的争议。我们描述了在三级儿科护理中心过敏/免疫科设立的复发性发热疾病诊所所涉及的疾病谱。最后,我们提供实用的建议,包括历史特征和初步实验室调查,可以帮助临床医生适当地管理这些患者。自身免疫性疾病或恶性肿瘤。为了应对这一挑战,我们回顾了发烧医学方法的历史、当前的诊断范式和管理中的争议。我们描述了在三级儿科护理中心过敏/免疫科设立的复发性发热疾病诊所所涉及的疾病谱。最后,我们提供实用的建议,包括历史特征和初步实验室调查,可以帮助临床医生适当地管理这些患者。

更新日期:2020-11-04
down
wechat
bug