以气道受累为主的复发性多软骨炎临床诊治进展

Advance in the clinical diagnosis and treatment of relapsing polychondritis with airway involvement

摘要:

复发性多软骨炎(RP)是一种以软骨及富含蛋白多糖的组织受累为主的免疫性多系统疾病，该病十分罕见，临床表现因受累部位不同而差异较大，目前仍无特异性辅助检查作为有效确诊手段，临床诊断较困难，以气道受累为主的患者，可隐匿起病，症状不典型，易误诊和漏诊，但气道受累时病情进展较快，晚期出现喉气管狭窄时治疗相当棘手且预后不佳，给气道管理带来了更大的挑战，因此针对气道受累的RP患者，临床医生应尽可能鉴别并提高早期诊断率，治疗上不仅要采取全身治疗，还要辅以局部治疗管理气道，本文就气道受累的复发性多软骨炎的诊治进展作一综述，为临床医师提供参考。

关键词:气道;复发性多软骨炎;诊断;治疗

Abstract:

Relapsing Polychondritis(RP) is a rare immune multisystem disease mainly involve cartilage and protein-polysaccharide-rich tissues. Clinical manifestations of RP are greatly different due to the different involved tissues. There is still no specific auxiliary examination to be used as a means of effective diagnosis, which makes clinical diagnosis difficult. Patients with airway involvement can be hidden onset, who are easy to misdiagnosis and missed diagnosis due to atypical symptoms. However, the disease progresses quickly when the airway is involved. The treatment of late laryngotracheal stenosis is quite difficult and the prognosis is poor, which brings greater challenges to airway management. Therefore, clinicians should identify and improve the early diagnosis rate of RP patients with airway involvement as much as possible, and take not only systemic treatment, but also local treatment to manage the airway. This article reviews the progress of diagnosis and treatment of Relapsing Polychondritis with airway involvement to provide reference for clinicians.

Key words:Airway;Relapsing polychondritis;Diagnosis;Treatment