Insidious airway involvement in patients with relapsing polychondritis / 中华风湿病学杂志

ABSTRACT

Objective To investigate clinical and imaging characteristics of patients with relapsing polychondritis (RP) with insidious airway involvement.Methods Data 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 MF ratio of 11.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 wail 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 (x2=15.580,P＜0.01),ocular involvement (x2=8.105,P＜0.01) were found to be more in patients with asymptomatic airway damage.All diagnosis before RP was organ-driven.Conclusion Airway 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.