Malard O, Hamidou M, Toquet C, Bailleuil S, Bordure Ph, Beauvillain De Montreuil C
Service d'O.R.L. et de chirurgie cervico-faciale, CHU Hôtel-Dieu, 1, place A. Ricordeau, BP 1005, 44093 Nantes Cedex 01, France.
Ann Otolaryngol Chir Cervicofac. 2002 Sep;119(4):202-8.
Relapsing polychondritis (RP) is a recurrent, chronic and rare disease of unknown etiology, considered as a systemic vasculitis. RP is characterized by inflammation of cartilaginous structures of the ears, nose, respiratory tract and joints. RP is likely initiated by ENT symptoms. Etiology is unknown but the association with HLA-DR4 and the occurrence of antibodies to type-II collagen suggest that an immunologic mechanism is involved in its pathogenesis. Diagnosis is difficult requiring identification of elastic cartilaginous injuries. Delay before diagnosis is usually important after the first attack. Neither serum investigation nor histological confirmation are necessary to establish the RP diagnosis, and ENT symptoms are generally sufficient to achieve the diagnosis. Prognosis is linked to laryngeal, tracheal and cardiovascular involvements. An association with myelodysplasia is acknowledged. Based on these three cases and data in the literature, we review classical diagnostic criteria (McAdam), prognosis and therapeutic outcome.
复发性多软骨炎（RP）是一种病因不明的复发性、慢性罕见疾病，被视为一种系统性血管炎。RP的特征是耳部、鼻部、呼吸道和关节的软骨结构发生炎症。RP可能由耳鼻喉科症状引发。病因不明，但与HLA - DR4的关联以及抗II型胶原抗体的出现表明其发病机制涉及免疫机制。诊断困难，需要识别弹性软骨损伤。首次发作后通常在诊断前会有较长延迟。建立RP诊断既不需要血清学检查也不需要组织学证实，耳鼻喉科症状通常足以确诊。预后与喉部、气管和心血管受累有关。RP与骨髓发育异常有关联。基于这三例病例以及文献中的数据，我们回顾了经典的诊断标准（麦克亚当标准）、预后和治疗结果。
