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[结节病的诊断与治疗。现行标准]

[Diagnosis and treatment of sarcoidosis. Current standards].

作者信息

Frye B C, Schupp J C, Köhler T L, Müller-Quernheim J

机构信息

Klinik für Pneumologie, Department Innere Medizin, Universitätsklinikum Freiburg, Killianstr. 5, 79106, Freiburg, Deutschland.

出版信息

Internist (Berl). 2015 Dec;56(12):1346-52. doi: 10.1007/s00108-015-3757-1.

Abstract

Sarcoidosis is a granulomatous disease that mainly affects the lungs and intrathoracic lymph nodes; however, virtually any organ can be affected. As an orphan disease, recommendations are mainly based on observational or small randomized studies as well as experts' opinion. Diagnosing sarcoidosis requires proof of non-necrotizing granulomas in patients with a compatible symptomatic pattern and the exclusion of other granulomatous diseases. Granulomas can be detected best in the lungs or intrathoracic lymph nodes. Therefore, bronchoscopy and endobronchial ultrasound with biopsies of lymph nodes are the major tools to diagnose sarcoidosis. Frequently, close follow-up and symptomatic therapy are sufficient to allow for spontaneous resolution. In case of functional organ impairment, cardial or CNS involvement, or other complications, steroid therapy is necessary with a starting dose of 0.5 mg/kg body weight that should be tapered-off over 6-12 months. Steroid-refractory disease can be treated by adding methotrexate or azathioprine, two drugs long known in sarcoidosis treatment. Monoclonal antibodies against TNF and lung transplantation are further therapeutic options.

摘要

结节病是一种肉芽肿性疾病,主要影响肺部和胸内淋巴结;然而,实际上任何器官都可能受累。作为一种罕见病,相关建议主要基于观察性研究或小型随机研究以及专家意见。诊断结节病需要在有符合症状模式的患者中证实存在非坏死性肉芽肿,并排除其他肉芽肿性疾病。肉芽肿在肺部或胸内淋巴结中最易被检测到。因此,支气管镜检查及经支气管超声引导下淋巴结活检是诊断结节病的主要手段。通常,密切随访和对症治疗足以使病情自发缓解。若出现器官功能损害、心脏或中枢神经系统受累或其他并发症,则需要使用类固醇治疗,起始剂量为0.5mg/kg体重,并应在6至12个月内逐渐减量。对于类固醇难治性疾病,可加用甲氨蝶呤或硫唑嘌呤进行治疗,这两种药物在结节病治疗中早已为人所知。抗TNF单克隆抗体和肺移植是其他治疗选择。

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