以气道受累为主的复发性多软骨炎患者的临床特征分析

doi:10.16138/j.1673-6087.2024.06.05

摘要/Abstract

摘要：

目的：探讨累及气道的复发性多软骨炎(relapsing polychondritis, RP)患者的临床特征。方法：对2007年7月至2023年12月在海军军医大学第一附属医院住院的141例RP患者临床资料进行回顾性分析。结果：RP患者气道受累的发生率为58.87%(83/141)，其中以气管壁增厚最常见，伴气道受累的RP患者确诊年龄更大（P=0.011），不伴气道受累的RP患者更易出现耳软骨、眼部和心血管系统受累（P=0.008、P=0.009和P=0.021）。不伴气道受累的RP患者超敏C反应蛋白（P=0.023）和血小板计数（P=0.013）显著高于伴气道受累的RP患者，伴气道受累的RP患者中性粒细胞计数高于不伴气道受累的RP患者（P=0.018）。单因素Logistic回归分析显示，确诊年龄和中性粒细胞计数与气道受累风险增加相关；耳软骨受累、眼部受累、心血管受累、红细胞沉降率、超敏C反应蛋白、血小板计数与丙氨酸转氨酶与气道受累风险降低相关。多因素Logistic回归分析证实确诊年龄大和中性粒细胞计数高是气道受累的独立危险因素。结论：RP患者气道受累与确诊年龄及中性粒细胞计数相关，建议定期行胸部CT检查，必要时行支气管检查。

关键词: 复发性多软骨炎, 气道受累, 临床特征

Abstract:

Objective To investigate the clinical characteristics of patients with relapsing polychondritis (RP) involving airway. Methods The clinical data of 141 RP patients hospitalized in the First Affiliated Hospital of Naval Medical University from July 2007 to December 2023 were analyzed retrospectively. Results The incidence of airway involvement in RP patients was 58.87%, among which airway wall thickening was the most common. RP patients with airway involvement were diagnosed at a later age (P=0.011). RP patients without airway involvement were more likely to have ear cartilage, eye and cardiovascular system involvement (P=0.008, P=0.009 and P=0.021); the hypersensitive C-reactive protein and platelet counts in RP patients without airway involvement were higher than those in RP patients with airway involvement (P=0.023, P=0.013), while the neutrophil counts in the patients were lower than those in RP patients with airway involvement (P=0.018). Univariate Logistic regression analysis showed that age at diagnosis and neutrophil count were positively correlated with airway involvement. In contrast, ear cartilage, eye and cardiovascular involvement, erythrocyte sedimentation rate, hypersensitive C-reactive protein, platelet count, and alanine transferase were negatively correlated with airway involvement. Multivariate Logistic regression analysis confirmed that age at diagnosis and neutrophil count were independent risk factors for airway involvement. Conclusions Airway involvement in RP patients is related to age at diagnosis and neutrophil count. The patients should receive regular chest CT and bronchial examination if necessary.

Key words: Relapsing polychondritis, Airway involvement, Clinical features

中图分类号:

R681.3

胡佳琪, 靳正逸, 刘齐龙, 吉连梅, 马泰彦, 高洁. 以气道受累为主的复发性多软骨炎患者的临床特征分析[J]. 内科理论与实践, 2024, 19(06): 379-384.

HU Jiaqi, JIN Zhengyi, LIU Qilong, JI Lianmei, MA Taiyan, GAO Jie. Relapsing polychondritis mainly with airway involvement: analysis of clinical characteristics[J]. Journal of Internal Medicine Concepts & Practice, 2024, 19(06): 379-384.

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参考文献 22

[1]	Padoan R, Campaniello D, Iorio L, et al. Biologic therapy in relapsing polychondritis: navigating between options[J]. Expert Opin Biol Ther, 2022, 22(5):661-671. doi: 10.1080/14712598.2022.2048647 pmid: 35230215
[2]	Cardoneanu A, Rezus II, Burlui AM, et al. Autoimmunity and autoinflammation: relapsing polychondritis and VEXAS syndrome challenge[J]. Int J Mol Sci, 2024, 25(4):2261.
[3]	徐健, 王丹丹, 石桂秀, 等. 复发性多软骨炎诊疗规范[J]. 中华内科杂志, 2022, 61(05):525-530.
[4]	Dion J, Costedoat-Chalumeau N, Sène D, et al. Relapsing polychondritis can be characterized by three different clinical phenotypes: analysis of a recent series of 142 patients[J]. Arthritis Rheumatol, 2016, 68(12):2992-3001.
[5]	Zhai SY, Guo RY, Zhang C, et al. Clinical analysis of relapsing polychondritis with airway involvement[J]. J Laryngol Otol, 2023, 137(1):96-100.
[6]	Jafarpour M, Saberivand M, Saemi M, et al. A multicenter study of long-term outcomes of relapsing polychondritis in Iran[J]. Sci Rep, 2024, 14(1):16486. doi: 10.1038/s41598-024-67530-8 pmid: 39020004
[7]	Damiani JM, Levine HL. Relapsing polychondritis—report of ten cases[J]. Laryngoscope, 1979, 89(6 Pt 1):929-946. pmid: 449538
[8]	McAdam LP, O’Hanlan MA, Bluestone R, et al. Relapsing polychondritis: prospective study of 23 patients and a review of the literature[J]. Medicine (Baltimore), 1976, 55(3):193-215.
[9]	Mertz P, Costedoat-Chalumeau N, Ferrada MA, et al. Relapsing polychondritis: clinical updates and new differential diagnoses[J]. Nat Rev Rheumatol, 2024, 20(6):347-360. doi: 10.1038/s41584-024-01113-9 pmid: 38698240
[10]	Liu Y, Cheng L, Zhao M, et al. Development and validation of diagnostic and activity-assessing models for relapsing polychondritis based on laboratory parameters[J]. Front Immunol, 2023, 14:1274677.
[11]	Kuo IC, Hsieh CI, Lee YC, et al. Diagnostic challenges and management of relapsing polychondritis with large-airway involvement: a case series and literature review[J]. Life (Basel), 2024, 14(9):1194.
[12]	张海艇, 王立, 闫琳毅, 等. 复发性多软骨炎患者临床特征分析[J]. 中华医学杂志, 2015 (29): 2375-2378.
[13]	Lin DF, Yang WQ, Zhang PP, et al. Clinical and prognostic characteristics of 158 cases of relapsing polychondritis in China and review of the literature[J]. Rheumatology international, 2016, 36: 1003-1009.
[14]	Rafeq S, Trentham D, Ernst A. Pulmonary manifestations of relapsing polychondritis[J]. Clin Chest Med, 2010, 31(3):513-518. doi: 10.1016/j.ccm.2010.04.004 pmid: 20692543
[15]	de Montmollin N, Dusser D, Lorut C, et al. Tracheobronchial involvement of relapsing polychondritis[J]. Autoimmun Rev, 2019, 18(9):102353.
[16]	Shimizu J, Yamano Y, Kawahata K, et al. Relapsing polychondritis patients were divided into three subgroups: patients with respiratory involvement (R subgroup), patients with auricular involvement (A subgroup), and overlapping patients with both involvements (O subgroup), and each group had distinctive clinical characteristics[J]. Medicine (Baltimore), 2018, 97(42):e12837.
[17]	Yin R, Xu D, Wang Q, et al. Predictors and prognosis of tracheostomy in relapsing polychondritis[J]. Rheumatology (Oxford), 2024, 63(11):3042-3049.
[18]	Jalaber C, Puéchal X, Saab I, et al. Differentiating tracheobronchial involvement in granulomatosis with polyangiitis and relapsing polychondritis on chest CT: a cohort study[J]. Arthritis Res Ther, 2022, 24(1):241. doi: 10.1186/s13075-022-02935-2 pmid: 36307863
[19]	Civan C, Işık EG, Has Şimşek D, et al. Diagnosis and evaluation of treatment response in relapsing polychondritis using 18F-FDG PET/CT[J]. Mol Imaging Radionucl Ther, 2024, 33(2):109-111.
[20]	Zhang L, Yun S, Wu T, et al. Clinical patterns and the evolution of relapsing polychondritis based on organ involvement: a Chinese retrospective cohort study[J]. Orphanet J Rare Dis, 2021, 16(1):225. doi: 10.1186/s13023-021-01861-x pmid: 34001193
[21]	蒋黎, 曹晓宇, 赵梦珠, 等. 呼吸道受累复发性多软骨炎患者的临床特点[J]. 中华临床免疫和变态反应杂志, 2021, 15(3):312-317.
[22]	刘禹. 哮喘气道中性粒细胞炎症表型的动态观察和临床意义研究[D]. 重庆: 第三军医大学, 2015.

项目	总RP患者（n=141)	气道受累组（n=83)	非气道受累组（n=58)	t/Z/χ²	P
女性[n/（%）]	69(48.9)	40（48.2）	29(50.0）	0.045	0.833
起病年龄（岁）	45.61±5.13	45.20±5.13	46.19±5.12	1.123	0.264
确诊年龄（岁）	50.27±5.10	51.18±4.85	48.97±5.22	-2.588	0.011
病程（月）	9.0（6.5，10.0）	9.0（7.0，10.0）	9.0（6.0，10.0）	-1.118	0.264
耳软骨受累[n（%）]	64(45.4)	30(36.1)	34(58.6)	6.958	0.008
鼻受累[n（%）]	73(51.8)	44(53.0)	29(50.0)	0.124	0.725
眼部受累[n（%）]	48(34.0)	21(25.3)	27(46.6)	6.867	0.009
前庭功能受累[n（%）]	8(5.7)	6(7.2)	2(3.4)	0.912	0.340
关节受累[n（%）]	108(76.6)	61(73.5)	47(81.0)	1.083	0.298
心血管受累[n（%）]	50(35.5)	23(27.7)	27(46.6)	5.296	0.021
肾脏受累[n（%）]	5(3.5)	3(3.6)	2(3.4)	0.003	0.958
神经系统受累[n（%）]	25(17.7)	14(16.9)	11(19.0)	0.103	0.748
红细胞沉降率（mm/h）	15.3(12.5，20.0)	14.5(12.1，19.3)	17.9(13.58，21.0)	-3.077	0.002
超敏C反应蛋白（mg/L）	16.4(13.5，20.4)	14.8(12.5，20.5)	18.6(15.3，20.5)	-2.281	0.023
血红蛋白(g/L)	112(105.5，121)	114(110，121)	107(104，121)	-1.961	0.05
白细胞计数(×10⁹/L)	6.86(5.74，9.45)	6.93(5.78，9.13)	6.46(5.23，9.63)	-0.633	0.527
中性粒细胞计数(×10⁹/L)	5.08±2.27	5.46±2.65	4.54±1.43	-2.398	0.018
血小板计数(×10⁹/L)	210.6±67.8	198.8±61.2	227.4±73.6	2.515	0.013
铁蛋白（μg/L）	142.0(73.0，196.5)	137.0 (75.0，196.0)	155.0 (59.0，201.5)	-0.547	0.584
丙氨酸转氨酶（U/L）	45.8±9.1	44.4±7.3	47.7±11.0	1.992	0.049
肌酐（μmol/L）	84.0 (73.5，95.0)	84.0(69.0，94.0)	85.5(74.0，97.5)	-1.717	0.086
ANA阳性[n（%）]	29(20.6)	18(21.7)	11(19.0)	0.155	0.694
ANCA阳性[n（%）]	16(11.3)	8(9.6)	8(13.8)	0.586	0.444

项目	OR(95%CI)	P
女性	0.931 (0.478~1.813)	0.833
起病年龄	0.963 (0.901~1.030)	0.264
确诊年龄	1.089 (1.020~1.163)	0.011
病程	0.963 (0.901~1.030)	0.264
耳软骨受累	0.398 (0.201~0.789)	0.008
鼻受累	1.127 (0.579~2.196)	0.725
眼部受累	0.389 (0.191~0.793)	0.009
前庭功能受累	2.167 (0.416~11.289)	0.340
关节受累	0.650 (0.289~1.463)	0.298
心血管受累	0.440 (0.219~0.884)	0.021
肾脏受累	1.051 (0.170~6.493)	0.958
神经系统受累	0.867 (0.363~2.070)	0.748
红细胞沉降率	0.424 (0.246~0.731)	0.002
超敏C反应蛋白	0.459 (0.234~0.900)	0.023
血红蛋白	1.003 (1.000~1.006)	0.050
白细胞计数	1.052 (0.895~1.236)	0.527
中性粒细胞计数	1.249 (1.039~1.501)	0.018
血小板计数	0.994 (0.989~0.999)	0.013
铁蛋白	0.999 (0.995~1.003)	0.584
丙氨酸转氨酶	0.961 (0.924~1.000)	0.049
肌酐	0.982(0.962~1.002)	0.086
ANA	1.183 (0.512~2.733)	0.694
ANCA	0.667 (0.236~1.884)	0.444

项目	OR(95%CI)	P
确诊年龄	1.097 (1.015~1.186)	0.020
耳软骨受累	0.352 (0.159~0.780)	0.010
眼部受累	0.401 (0.182~0.885)	0.024
心血管受累	0.468 (0.219~0.999)	0.049
红细胞沉降率	0.457 (0.251~0.832)	0.010
超敏C反应蛋白	0.512 (0.245~1.069)	0.074
中性粒细胞计数	1.215 （1.001~1.474）	0.048
血小板计数	0.995 (0.990~1.001)	0.082
丙氨酸转氨酶	0.971 (0.931~1.013)	0.173