Department of Pneumology, Assistance Publique Hôpitaux de Paris, Avicenne University Hospital, Bobigny, France; University Paris 13, Sorbonne Paris Cité, Bobigny, France.
Department of Pneumology, University Hospital, Freiburg, Germany.
Lancet. 2014 Mar 29;383(9923):1155-67. doi: 10.1016/S0140-6736(13)60680-7. Epub 2013 Oct 1.
Sarcoidosis is a systemic disease of unknown cause that is characterised by the formation of immune granulomas in various organs, mainly the lungs and the lymphatic system. Studies show that sarcoidosis might be the result of an exaggerated granulomatous reaction after exposure to unidentified antigens in individuals who are genetically susceptible. Several new insights have been made, particularly with regards to the diagnosis and care of some important manifestations of sarcoidosis. The indications for endobronchial ultrasound in diagnosis and for PET in the assessment of inflammatory activity are now better specified. Recognition of unexplained persistent disabling symptoms, fatigue, small-fibre neurological impairment, cognitive failure, and changes to health state and quality of life, has improved. Mortality in patients with sarcoidosis is higher than that of the general population, mainly due to pulmonary fibrosis. Predicted advances for the future are finding the cause of sarcoidosis, and the elucidation of relevant biomarkers, reliable endpoints, and new efficient treatments, particularly in patients with refractory sarcoidosis, lung fibrosis, and those with persistent disabling symptoms.
结节病是一种病因不明的全身性疾病，其特征是在各种器官中形成免疫肉芽肿，主要是肺部和淋巴系统。研究表明，结节病可能是遗传易感个体暴露于未知抗原后发生过度肉芽肿反应的结果。特别是在一些重要结节病表现的诊断和治疗方面，有了一些新的认识。目前，支气管内超声在诊断中的应用以及 PET 在炎症活动评估中的应用指征更加明确。对不明原因的持续性致残症状、疲劳、小纤维神经损伤、认知功能障碍以及健康状态和生活质量变化的认识有所提高。结节病患者的死亡率高于普通人群，主要是由于肺纤维化所致。未来的预期进展是找到结节病的病因，阐明相关的生物标志物、可靠的终点和新的有效治疗方法，特别是在难治性结节病、肺纤维化和持续性致残症状患者中。
