38.3 原发性免疫缺陷：何时不仅仅是 "JIA"。

IF 4.5 2区医学 Q1 RHEUMATOLOGY Best Practice & Research in Clinical Rheumatology Pub Date : 2024-09-01 DOI:10.1016/j.berh.2024.101960

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引用次数: 0

摘要

青少年特发性关节炎（JIA）有时被认为是一种排除性诊断，因为它的名称意味着这种关节炎没有明显的病因。尽管如此，JIA 仍有一些经典的临床特征，并根据表型定义了许多类别。由于没有针对 JIA 的诊断测试，包括原发性免疫缺陷症（PID）在内的可模拟 JIA 的疾病有时会被误诊为 JIA。怀疑 PID 的线索包括发病年龄早、有家族史、对感染的易感性增加、荨麻疹等异常特征、间质性肺病、感音神经性听力损失和对常规治疗反应差等。本综述将重点介绍 PID 的基本知识，并讨论可能伴有关节炎从而与 JIA 相混淆的 PID。

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38.3 Primary Immunodeficiencies: When is it not just “JIA”

Juvenile Idiopathic Arthritis (JIA) is sometimes considered a diagnosis of exclusion as the name signifies that no cause is evident for this form of arthritis. Despite this JIA has some classical clinical features and many categories are defined based on the phenotype. Since there is no diagnostic test for JIA, diseases that can mimic JIA, including Primary Immunodeficiencies (PID) can sometimes be misdiagnosed as JIA. The clues to suspecting PIDs are early age of onset, presence of family history, increased susceptibility to infections, unusual features like urticaria, interstitial lung disease, sensorineural hearing loss and poor response to conventional therapy, amongst others. This review will highlight the basics of PIDs and will discuss PIDs that can present with arthritis and hence can be confused with JIA.

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来源期刊

Best Practice & Research in Clinical Rheumatology 医学-风湿病学

CiteScore

9.40

自引率

0.00%

发文量

审稿时长

27 days

期刊介绍： Evidence-based updates of best clinical practice across the spectrum of musculoskeletal conditions. Best Practice & Research: Clinical Rheumatology keeps the clinician or trainee informed of the latest developments and current recommended practice in the rapidly advancing fields of musculoskeletal conditions and science. The series provides a continuous update of current clinical practice. It is a topical serial publication that covers the spectrum of musculoskeletal conditions in a 4-year cycle. Each topic-based issue contains around 200 pages of practical, evidence-based review articles, which integrate the results from the latest original research with current clinical practice and thinking to provide a continuous update. Each issue follows a problem-orientated approach that focuses on the key questions to be addressed, clearly defining what is known and not known. The review articles seek to address the clinical issues of diagnosis, treatment and patient management. Management is described in practical terms so that it can be applied to the individual patient. The serial is aimed at the physician in both practice and training.