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周期性发热、口疮性口炎、咽炎和淋巴结炎综合征和儿童及成人体征不明性反复发热综合征。

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome and Syndrome of Unexplained Recurrent Fevers in Children and Adults.

机构信息

Department of Medicine, Harvard Medical School, Boston, Mass; Program in Rheumatology, Division of Immunology, Boston Children's Hospital, Boston, Mass; Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, Boston, Mass.

Department of Medicine, Harvard Medical School, Boston, Mass; Department of Pediatrics, Harvard Medical School, Boston, Mass.

出版信息

J Allergy Clin Immunol Pract. 2023 Jun;11(6):1676-1687. doi: 10.1016/j.jaip.2023.03.014. Epub 2023 Mar 21.

DOI:10.1016/j.jaip.2023.03.014
PMID:36958521
Abstract

Children and adults with autoinflammatory disorders, who often experience recurrent fevers, rashes, cold-induced symptoms, conjunctivitis, lymphadenopathy, recurrent infections, aphthous stomatitis, and abnormal blood cell counts, may present to the allergist/immunologist because the symptoms mimic allergies and disorders of immunity. In recent years, there has been increased recognition of non-monogenic autoinflammatory disorders, including periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome and syndrome of undifferentiated recurrent fevers. For many clinical practitioners, the natural history, diagnostic criteria, differential diagnoses, and preferred therapies remain challenging because of the presumed rarity of patients and the evolving field of autoinflammation. Here, we aim to provide a practical framework for the clinical allergist/immunologist to evaluate and treat this patient population.

摘要

患有自身炎症性疾病的儿童和成人经常会出现反复发作的发热、皮疹、冷诱导症状、结膜炎、淋巴结病、反复感染、口疮性口炎和血细胞计数异常,可能会因为症状类似于过敏和免疫紊乱而就诊于过敏症/免疫学家。近年来,人们对非单基因自身炎症性疾病的认识有所增加,包括周期性发热、口疮性口炎、咽炎和淋巴结炎综合征和未分化复发性发热综合征。对于许多临床医生来说,由于患者的假定罕见性和自身炎症领域的不断发展,其自然病史、诊断标准、鉴别诊断和首选治疗方法仍然具有挑战性。在这里,我们旨在为临床过敏症/免疫学家提供一个实用的框架,以评估和治疗这一患者群体。

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