复发性多软骨炎的隐匿性气道受累临床分析

Insidious airway involvement in patients with relapsing polychondritis

目的探讨复发性多软骨炎（RP）伴隐匿性气道受累的临床及影像特征和病情评价。方法连续录入可疑RP患者，明确诊断并病情评价和分组分析伴及不伴气道受累患者的相关临床及影像资料。满足正态分布计量资料的2组间比较采用t检验；不满足正态分布计量资料采用Mann-Whitney U检验；定性资料采用χ^2检验。结果在2007年12月1日至2016年7月31日共收集到资料齐全且符合Michet诊断标准的RP患者216例，男性108例，女性108例；发病年龄8-86岁，平均（44±16）岁；确诊年龄13-87岁，平均（47±15）岁；病程时间中位数12（0.3-480）个月；RP患者疾病活动指数（RPDAI）为35（8-67）；RP患者器官损伤指数（RPODI）为2.4（0.1-84）；前3位常见的起病类型分别是耳廓软骨炎（28.7%）、气道软骨炎（24.1%）及眼睛受累（22.2%）；RPODI在耳廓软骨炎起病型（4.4）高于中位数（2.4）（Z=-2.084，P〈0.05）、在鼻软骨炎起病型（0.6）明显低于中位数（2.4）（Z=-2.425，P〈0.05）。高达81.5%的患者伴气道损害，其中52.3%无症状；常见的气道损害部位是主气管（79.0%，139/176）；常见的气道受累的CT表现为气管壁增厚（72.7%，128/176）；气道管腔狭窄多见于伴气道症状者；气管壁钙化多见于无气道症状者；耳廓软骨炎（χ^2=15.580，P〈0.01）及眼受累（χ^2=8.105，P〈0.01）多发生在无气道损害者，而听力下降及鼻软骨炎的发生与气道损害无关；RP确诊前的诊断均与起病类型及感染相关；结论RP累及气道为常见3个起病类型之一，且半数以上无症状；RPODI与RP器官损伤程度及病程有关；常规气道影像学所见（管壁增厚、钙化及狭窄）有助于RP的早期发现。

ObjectiveTo investigate clinical and imaging characteristics of patients with relapsing polychondritis （RP） with insidious airway involvement.MethodsData collected prospectively and consecutively for patients with the diagnosis of RP and their disease activity evaluation was analyzed. The t-test and Mann-Whitney U test were used for statistical analysis between the two groups forquantitative datain normal distri-bution and non-normal distribution respectively, while Chi-square test was use for qualitative data analysis.Results Two hundred and sixteen patients with complete data from Dec 1, 2007 to Jul 31, 2016 were enrolled with a M∶F ratio of 1∶1. Mean age of disease onset was （44±16）（8-86） years. The median disease duration was 12 （0.3-480） month. The median relapsing polychondritis disease activity index （RPDAI） was 35 （8-67）, the median RPODI was 2.4 （0.1-84）. The top three initial presentation were auricular chondritis （28.7%）, airway chondritis （24.1%） and ocular involvement （22.2%） respectively. RPODI was significantly higher in auricular chondritis （4.4） （Z=-2.084, P〈0.05） and lower in nose chondritis （0.6） （Z=-2.425, P〈0.05） . Up to 81.5% of the patients were found with airway damage and 52.3% of them were asymptomatic. Airway damage was mostly located in trachea （79.0% 139/176） and common features on CT scan werecharacterized by airway wall thickening （72.7%, 128/176）. Airway narrowing was mostly seen in symptomatic patients while calcification was seen more in asymptomatic patients. Insidious hearing-loss and nose chondritis were found not related to airway damage while obvious auricular chondritis （χ^2=15.580, P〈0.01）, ocular involvement （χ^2=8.105, P〈0.01） were found to be more in patients with asymptomatic airway damage. All diagnosis before RP was organ-driven.ConclusionAirway involvement in RP is one of the three most common disease initial presentation-sand half of them are asymptomatic. RPODI is a reasonable marker for disease evaluation. Routine follow-up of airway damage （wall thickness, calcification and lumen narrowing） is essential for early RP recognition.