Pelzer Theo, Jung Pius
Dtsch Med Wochenschr. 2021 Mar;146(5):335-343. doi: 10.1055/a-1239-4492. Epub 2021 Mar 1.
Sarcoidosis occurs predominantly in younger adults and may involve multiple organ systems. Although classical features such as bihilar lymphadenopathy, low grade fever, fatigue, pulmonary opacities, ocular lesions and arthritis occur frequently it must be kept in mind that sarcoidosis is able to mimic virtually any other disease. A thorough and systematic diagnostic strategy is warranted since even the histological hallmark of non-caseating granulomas occurs in a variety of other granulomatous diseases and even malignancies. A firm diagnosis is based on a match of clinical features, imaging results and histopathological findings. The Scadding classification describes four different types, not stages, of thoracic sarcoidosis based on mediastinal lymph node and lung parenchyma involvement. Medical treatment of pulmonary sarcoidosis is indicated only in patients exhibiting progressive disease and/or organ function impairment. Cardiac sarcoidosis manifests itself by clinical signs of heart failure, impaired cardiac function and arrhythmias including ventricular tachycardia, ventricular fibrillation and AV-conduction abnormalities. Patients with symptomatic or suspected cardiac sarcoidosis require rapid and elaborate diagnostic testing including cardiac MRI and PET imaging. Referral to a specialized center should be considered to establish a firm diagnosis and to initiate medical treatment and eventual device implantation. Oral corticosteroids, as the initial medical treatment of choice, carry side effects that must be weighed carefully against clinical benefits. Immunosuppressive therapy with methotrexate, azathioprine or TNF-blockers is usually reserved for patients that are either not responsive or intolerant to systemic steroids or that require steroid maintenance therapy above the cushing threshold. Diagnosed early and treated correctly, pulmonary and cardiac sarcoidosis mostly carry a favorable prognosis.
结节病主要发生于年轻人,可能累及多个器官系统。尽管双肺门淋巴结肿大、低热、乏力、肺部阴影、眼部病变和关节炎等典型特征较为常见,但必须牢记,结节病几乎能够模仿任何其他疾病。由于非干酪样肉芽肿的组织学特征也见于多种其他肉芽肿性疾病甚至恶性肿瘤,因此需要采用全面系统的诊断策略。明确诊断基于临床特征、影像学结果和组织病理学发现的匹配。Scadding分类根据纵隔淋巴结和肺实质受累情况描述了胸内结节病的四种不同类型,而非阶段。仅在出现疾病进展和/或器官功能损害的患者中才考虑对肺部结节病进行药物治疗。心脏结节病表现为心力衰竭、心功能受损和心律失常的临床体征,包括室性心动过速、心室颤动和房室传导异常。有症状或疑似心脏结节病的患者需要进行快速而详尽的诊断检查,包括心脏MRI和PET成像。应考虑转诊至专业中心以明确诊断、开始药物治疗并最终进行器械植入。口服糖皮质激素作为首选的初始药物治疗,其副作用必须与临床获益仔细权衡。甲氨蝶呤、硫唑嘌呤或肿瘤坏死因子阻滞剂等免疫抑制治疗通常适用于对全身性激素无反应或不耐受,或需要超过库欣阈值的激素维持治疗的患者。早期诊断并正确治疗,肺部和心脏结节病大多预后良好。