| Objective:To investigate the clinical characteristics of relapsing polychondritis(RP)patients,and compare the sensitivity and specificity among McAdam,Damiani-Levine and Michet classification criteria.Methods:The clinical records of relapsing polychondritis(RP)patients,hospitalized at Peking Union Medical College Hospital between December 2005 and September 2019,were retrospectively collected.Patients diagnosed with other rheumatic diseases,sinusitis,tympanitis,systemic amyloidosis and diffusive panbronchiolitis were recruited as disease controls.McAdam,Damiani-Levine and Michet criteria were applied to the cohort and sensitivity and specificity were analyzed.Results:A total of 212 RP patients were hospitalized at Peking Union Medical College Hospital between December 2005 and September 2019.Nineteen patients complicated with other connective tissue diseases,systemic vasculitis,spondyloarthropathy,solid tumors,common variant immunodeficiency disease,myelodysplastic syndrome,and tuberculosis were excluded.193 RP patients and 130 control patients were eventually recruited.The sensitivity of Damiani-Levine criteria(89.1%)exceeded McAdam criteria(72.0%)(p<0.001)and Michet criteria(79.3%)(p<0.001)and the sensitivity of Michet criteria was higher than McAdam criteria(p<0.001).The specificity of McAdam and Michet criteria were 96.2%and higher than Damiani-Levine criteria(91.5%)(p=0.031).Conclusion:Relative to McAdam criteria and Michet criteria,DamianiLevine criteria had higher sensitivity and lower specificity.Objective:To investigate the clinical characteristics of RP patients with respiratory tract or cardiovascular involvement.Methods:The clinical manifestations and laboratory examination of RP patients,hospitalized at Peking Union Medical College Hospital between December 2005 and September 2019,were retrospectively reviewed.Cases which were incomplete or overlapped with other diseases that may interfere with the results were excluded.All patients were grouped according to respiratory or cardiovascular involvement,and the demographic characteristics,clinical manifestations,and laboratory results were compared between the involved and non-involved groups.Results A total of 193 patients were included to explore the clinical characteristics of RP patients with respiratory tract involvement.149 patients(77.2%)presented respiratory tract involvement.Among them,tracheal thickening was the most common in 123 cases(account for 82.6%of the respiratory tract involvement),followed by bronchomalacia(100,67.1%),tracheomalacia(80,53.7%)and subglottic stenosis(28,18.8%).Compared with RP patients without respiratory tract involvement,those with respiratory tract involvement were younger at the time of disease onset(43.3±11.1 years VS 49.9±14.6 years,P=0.014),experienced the longer time of diagnosis delay(median[IQR],8[4-14]months VS 5[2.2511]months,P=0.044)and had a higher incidence of thoracic costal cartilage involvement.The multiple factors Logistic models showed that risk factors of respiratory tract involvement was costochondral joint involvement(OR=2.9[1.1-7.4],P=0.031).Except for some RP patients with incomplete clinical data,150 patients were included in the analysis of cardiovascular involvement.42 patients(28.0%)presented cardiovascular involvement.The male to female ratio is 1:0.75.The mean age of RP onset was 50.8±11.3 years,the duration of disease was 10.5(4.3-24.8)months,and the delay to diagnosis was 7(4-14.6)months.Among them,aortic regurgitation was the most common in 22 cases(account for 52.4%of the cardiovascular involvement).RP patients with cardiovascular involvement had a higher incidence of auricular cartilage(71.4%VS 46.3%,P=0.006)and extremities joint(85.7%VS 67.6%,P=0.025)involvement.The multiple factors Logistic models showed that risk factors of cardiovascular involvement was extremities joint involvement(OR=2.8[1.0-7.7],P=0.044).After treatment with glucocorticoid combined with immunosuppressant,4 patients(account for 9.5%of the cardiovascular involvement)remained stable,5 patients(11.9%)died,and 33 patients(78.6%)improved.Conclusions RP patients with respiratory tract involvement were more likely occurred to younger people with delayed diagnosis and more frequent thoracic costal cartilage involvement.RP patients with cardiovascular involvement have a later onset and are more likely to have auricular cartilage and extremities joint involvement.Glucocorticoid combined immunosuppressive therapy is effective in most patients. |
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