School of Medical Education, King's College London, UK.
Louise Coote Lupus Unit, Guy's Hospital, London, UK.
Rheumatology (Oxford). 2018 Sep 1;57(9):1525-1532. doi: 10.1093/rheumatology/kex406.
Relapsing polychondritis (RPC) is a rare autoimmune rheumatic disorder that is traditionally classified as a systemic vasculitis. It is characterized by inflammation of cartilage, and typical presenting features include chondritis of the nasal bridge, auricular chondritis, ocular inflammation and involvement of the bronchial tree. Its rarity often leads to considerable delay in establishing a diagnosis and poses a significant management challenge to clinicians, as no conventional guidelines exist. This review summarizes the clinical features of RPC and provides guidance for rheumatologists on making the diagnosis and assessing organ involvement. The current state of RPC management is reviewed, with a focus on the use of the anti-TNF-α agents in patients with pulmonary involvement, the leading cause of mortality and morbidity in RPC.
复发性多软骨炎(RPC)是一种罕见的自身免疫性风湿性疾病,传统上被归类为系统性血管炎。其特征为软骨炎症,典型的表现特征包括鼻梁软骨炎、耳软骨炎、眼部炎症和支气管树受累。由于其罕见性,常常导致诊断的显著延迟,并对临床医生提出了重大的管理挑战,因为目前没有常规的指导方针。本综述总结了 RPC 的临床特征,并为风湿病医生提供了诊断和评估器官受累的指导。还回顾了 RPC 管理的现状,重点关注抗 TNF-α 药物在肺部受累患者中的应用,肺部受累是 RPC 导致死亡率和发病率的主要原因。