Departement of Chest Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France.
National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP-HP, Université Paris Descartes, Paris, France.
Autoimmun Rev. 2019 Sep;18(9):102353. doi: 10.1016/j.autrev.2019.102353. Epub 2019 Jul 16.
Recent studies show that relapsing polychondritis patients with tracheobronchial involvement are distinct from others in terms of clinical characteristics, therapeutic management, and disease evolution. Tracheobronchial involvement affects 20 to 50% of patients and may reveal the disease. It should be sought at the time of diagnosis and at each follow-up visit. Respiratory impairment is confirmed by computed tomography (CT) of the chest, including the cervical portion of the trachea, with end-inspiratory and dynamic expiratory scans, and pulmonary function tests. These investigations should be performed, even in asymptomatic patients, at the time of diagnosis, and repeated as necessary during follow-up. Bronchoscopy and a fortiori endoscopic intervention should be considered with caution and performed only by expert endoscopists after careful evaluation of the risks and benefits of such procedures, which can lead to damage or perforation of the airways and bronchospasm. Early detection and management of tracheobronchial involvement in relapsing polychondritis has significantly improved the prognosis of patients, especially with the development of interventional fiberoptic bronchoscopy. However, relapsing polychondritis-related morbidity and mortality are still elevated, particularly in tracheobronchial disease.
最近的研究表明,复发性多软骨炎伴有气管支气管受累的患者在临床特征、治疗管理和疾病进展方面与其他患者不同。气管支气管受累影响 20%至 50%的患者,可能会导致疾病的出现。在诊断时以及每次随访时都应寻找气管支气管受累的迹象。呼吸功能损害通过胸部 CT (包括气管的颈部部分)证实,包括吸气末和动态呼气扫描以及肺功能检查。即使在无症状患者中,也应在诊断时进行这些检查,并根据需要在随访期间重复进行。支气管镜检查,更不用说内镜介入,应慎重考虑,并且仅在仔细评估这些程序的风险和收益后,由经验丰富的内镜医生进行,因为这些程序可能导致气道损伤或穿孔和支气管痉挛。早期发现和治疗复发性多软骨炎的气管支气管受累显著改善了患者的预后,尤其是在介入性纤维支气管镜技术发展之后。然而,复发性多软骨炎相关的发病率和死亡率仍然很高,尤其是在气管支气管疾病中。