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[结节病和铍中毒]

[Sarcoidosis and berylliosis].

作者信息

Soriano Daniel, Quartucci Caroline, Agarwal Prerana, Müller-Quernheim Joachim, Frye Björn Christian

机构信息

Klinik für Pneumologie, Universitätsklinikum Freiburg, Medizinische Fakultät, Albert-Ludwigs-Universität Freiburg, Killianstraße 5, 79106, Freiburg, Deutschland.

Institut und Poliklinik für Arbeits‑, Sozial- und Umweltmedizin, Klinikum der Universität München, LMU München, München, Deutschland.

出版信息

Internist (Berl). 2022 May;63(5):557-565. doi: 10.1007/s00108-022-01323-x. Epub 2022 Apr 9.

Abstract

Sarcoidosis and berylliosis (chronic beryllium disease, CBD) are granulomatous diseases and are phenocopies which cannot be differentiated based on the clinical presentation. Whereas for sarcoidosis the eliciting agent is unknown, for berylliosis an exposure to beryllium (mostly as occupational exposure) can be confirmed that therefore induces a sensitization against beryllium. The diagnosis is generally made in patients with a typical clinical presentation, the histological proof of a non-necrotizing granuloma and the exclusion of other diseases causing granulomas. In most cases, granulomas can be detected in the lungs and/or (intrathoracic) lymph nodes. The proof of sensitization to beryllium for the differential diagnosis can be performed with a so-called beryllium lymphocyte proliferation test in peripheral mononuclear blood cells or cells from a bronchoalveolar lavage. The objectives of treatment are avoidance of functional organ impairment and symptom control. Immunosuppressive therapy (initially mostly with corticosteroids) and supportive measures can prove beneficial; however, in many cases clinical observation can be sufficient because of stable disease or spontaneous resolution. In addition, further beryllium exposure must be avoided, which mostly necessitates a change of the workplace.

摘要

结节病和铍中毒(慢性铍病,CBD)是肉芽肿性疾病,是表型相似的疾病,无法根据临床表现进行区分。结节病的诱发因素尚不清楚,而铍中毒可确认有铍暴露(主要是职业暴露),因此会引发对铍的致敏反应。诊断通常基于典型的临床表现、非坏死性肉芽肿的组织学证据以及排除其他导致肉芽肿的疾病。在大多数情况下,肺部和/或(胸内)淋巴结可检测到肉芽肿。鉴别诊断时,可通过所谓的铍淋巴细胞增殖试验检测外周血单个核细胞或支气管肺泡灌洗细胞对铍的致敏情况。治疗目标是避免功能器官损害和控制症状。免疫抑制治疗(最初大多使用皮质类固醇)和支持措施可能有益;然而,在许多情况下,由于病情稳定或自行缓解,临床观察可能就足够了。此外,必须避免进一步接触铍,这大多需要更换工作场所。

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