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结节病的诊断问题。

Diagnosis issues in sarcoidosis.

机构信息

Inserm UMR 1272, université Paris 13, 93000 Bobigny, France; Service de pneumologie, hôpital Avicenne, AP-HP, 93000 Bobigny, France.

Inserm UMR 1272, université Paris 13, 93000 Bobigny, France; Service de pneumologie, hôpital Avicenne, AP-HP, 93000 Bobigny, France.

出版信息

Respir Med Res. 2020 Mar;77:37-45. doi: 10.1016/j.resmer.2019.09.002. Epub 2019 Oct 28.

DOI:10.1016/j.resmer.2019.09.002
PMID:32035337
Abstract

Multiple problems may be encountered during the diagnosis of sarcoidosis: at first diagnose sarcoidosis in an appropriate clinical setting, secondly, identify any manifestation to be linked to sarcoidosis at diagnosis work-up and during evolution; thirdly, recognize "danger" in sarcoidosis and parasarcoidosis syndromes, and finally, diagnose sarcoidosis recovery. Diagnosis is often delayed as presentation may be diverse, non-specific, or atypical. Diagnosis of sarcoidosis is based on three criteria: a compatible presentation; evidence of non-caseating granulomas and exclusion of any alternative diagnosis. However, even when all criteria are fulfilled, the probability of sarcoidosis diagnosis varies from definite to only possible depending upon the presence of more or less characteristic radio-clinical and histopathological findings and on the epidemiological context. Bilateral hilar lymphadenopathy and/or diffuse lung micronodules mainly along lymphatics are the most frequent highly suggestive findings. Evidence of granulomas relies on superficial biopsies of clinically suspected lesion when present or most often by bronchial endoscopy. The diagnosis of sarcoidosis may be difficult in absence of thoracic or skin manifestations and may require the benefit of hindsight before being definitive. Differential diagnoses, mainly tuberculosis, must be considered. The diagnosis of events during evolution relies on serial clinical, pulmonary function, radiographic evaluation and on extrapulmonary manifestations work-up, including electrocardiogram and blood biology. Affected organs need to be related to sarcoidosis using an appropriate diagnostic assessment instrument. To declare the recovery of sarcoidosis, all manifestations must have disappeared spontaneously or after 3-5 years post-treatment without relapse.

摘要

在诊断结节病时可能会遇到多种问题

首先,在适当的临床环境中诊断结节病;其次,在诊断性检查和疾病进展过程中,识别任何与结节病相关的表现;再次,认识到结节病和类结节病综合征中的“危险”信号;最后,诊断结节病的恢复。由于表现可能多种多样、非特异性或不典型,因此诊断往往会被延误。结节病的诊断基于三个标准:相符的表现;非干酪样肉芽肿的证据和排除任何其他诊断。然而,即使满足了所有标准,根据更具特征性的放射-临床和组织病理学发现以及流行病学背景,结节病诊断的可能性从确定到仅可能不等。双侧肺门淋巴结肿大和/或弥漫性肺微结节,主要沿淋巴管分布,是最常见的高度提示性表现。肉芽肿的证据依赖于存在临床疑似病变时进行的浅表活检,或者最常见的是通过支气管内镜进行的活检。在没有胸内或皮肤表现的情况下,结节病的诊断可能很困难,并且可能需要在明确诊断之前进行回顾性分析。必须考虑主要是结核病在内的鉴别诊断。疾病进展过程中的诊断依赖于连续的临床、肺功能、影像学评估以及对肺外表现的检查,包括心电图和血液生物学检查。受累器官需要使用适当的诊断评估工具与结节病相关联。要宣布结节病的恢复,所有表现都必须自发消失或在治疗后 3-5 年内无复发。

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