全身型幼年特发性关节炎合并肺受累临床特征及相关因素分析

doi:10.12372/jcp.2022.22e0462

Abstract

Abstract:

Objective To investigate the clinical characteristics and related factors of systemic juvenile idiopathic arthritis (sJIA) complicated with lung involvement. Methods The clinical data of children who were initially diagnosed with sJIA from January 2017 to January 2022 were retrospectively analyzed. Results A total of 73 children (40 boys and 33 girls) with sJIA were enrolled and the median age was 5.6 (3.1-8.8) years. There were 32 children in sJIA group without lung involvement and 41 children in sJIA group with lung involvement. Compared with sJIA group without lung involvement, the proportion of sJIA combined with macrophage activation syndrome, fatigue, chest tightness and rapid shallow breathing was higher, the level of IL-18 was higher, and the proportion of positive changes in chest radiography and lung high-resolution CT was higher, and the differences were statistically significant (P<0.05). The results of binary logistics regression analysis showed that fatigue, chest tightness, positive changes of chest radiograph and lung high-resolution CT, and increased IL-18 level were independent risk factors for sJIA complicated with lung involvement (P<0.05). Conclusion Early clinical symptoms, serum IL-18 level and pulmonary imaging examination have certain predictive value for the occurrence of sJIA complicated with lung involvement.

Key words: systemic juvenile idiopathic arthritis, lung involvement, clinical characteristics, related factor

TAN Xiaohua, LI Caifeng, ZHAO Wenjia, KUANG Weiying, DENG Jianghong, ZHANG Junmei. Clinical characteristics and related factors of pulmonary involvements in patients with systemic juvenile idiopathic arthritis[J].Journal of Clinical Pediatrics, 2022, 40(11): 864-868.

Figures/Tables 3

References 20

[1]	Martini A, Ravelli A, Avcin T, et al. Toward new classification criteria for juvenile idiopathic arthritis: first steps, Pediatric Rheumatology International Trials Organization International Consensus[J]. J Rheumatol, 2019, 46(2): 190-197. doi: 10.3899/jrheum.180168 pmid: 30275259
[2]	郦琳琳, 梁慧, 姚劲, 等, 儿童脱屑性间质性肺炎 1 例报告并文献复习[J]. 临床儿科杂志, 2020, 38(8): 612-615.
[3]	Suzuki T, Trapnell BC. Pulmonary alveolar proteinosis syndrome[J]. Clin Chest Med, 2016, 37(3): 431-40. doi: 10.1016/j.ccm.2016.04.006 pmid: 27514590
[4]	PettyRE,Southwood TR, Manners P, et al. International League of Associations for Rheumatology classification of juvenile idiopathic arthritis second revision,Edmonton,2001[J]. J Rheumatol, 2004, 31(2): 390-392. pmid: 14760812
[5]	周蔚然, 刘雪梅, 董琳琳, 等, 川崎病与幼年特发性关节炎全身型合并巨噬细胞活化综合征特点对比分析[J]. 临床儿科杂志, 2020, 38(10): 744-748.
[6]	Schulert GS, Yasin S, Carey B, et al. Systemic juvenile idiopathic arthritis-associated lung disease: characterization and risk factors[J]. Arthritis Rheumatol, 2019, 71(11): 1943-1954. doi: 10.1002/art.41073
[7]	Filocamo G, Davì S, Pistorio A, et al. Evaluation of 21-numbered circle and 10-centimeter horizontal line visual analog scales for physician and parent subjective ratings in juvenile idiopathic arthritis[J]. J Rheumatol, 2010, 37(7): 1534-1541. doi: 10.3899/jrheum.091474 pmid: 20551105
[8]	Singh G, Athreya BH, Fries JF, et al. Measurement of health status in children with juvenile rheumatoid arthritis[J]. Arthritis Rheum, 1994, 12(37) : 1761-1769.
[9]	Consolaro A, Ruperto N, Bazso A, et al. Development and validation of a composite disease activity score for juvenile idiopathic arthritis[J]. Arthritis Rheum, 2009, 61(5): 658-666. doi: 10.1002/art.24516
[10]	Saper VE, Chen G, Deutsch GH, et al, Emergent high fatality lung disease in systemic juvenile arthritis[J]. Ann Rheum Dis, 2019, 78(12): 1722-1731. doi: 10.1136/annrheumdis-2019-216040 pmid: 31562126
[11]	Erkens R, Esteban Y, Towe C, et al. Pathogenesis and treatment of refractory disease courses in systemic juvenile idiopathic arthritis: refractory arthritis, recurrent macrophage activation syndrome and chronic lung disease[J]. Rheum Dis Clin North Am, 2021, 47(4): 585-606. doi: 10.1016/j.rdc.2021.06.003
[12]	Sato S, Hosokawa T, Kawashima H. Successful treatment of plasma exchange for refractory systemic juvenile idiopathic arthritis complicated with macrophage activation syndrome and severe lung disease[J]. Ann Rheum Dis, 2022, 81(4): e61. doi: 10.1136/annrheumdis-2020-217390
[13]	Chen G, Deutsch GJ, Schulert G, et al, Serum proteome analysis of systemic JIA and related lung disease identifies distinct inflammatory programs and biomarkers[J]. Arthritis Rheumatol, 2022.doi:10.1002/art.42099. doi: 10.1002/art.42099
[14]	刘小惠, 王智, 蔡素芬, 等. 儿童风湿性疾病合并肺间质病变16例临床分析[J]. 江西医药, 2019, 54(8): 951-952.
[15]	南楠, 李丹, 冯媛, 等. 儿童结缔组织病并间质性肺病53例临床分析[J]. 中华实用儿科临床杂志, 2022, 37(1): 6.
[16]	Kudela H, Drynda S, Lux A, et al. Comparative study of Interleukin-18 (IL-18) serum levels in adult onset Still’s disease (AOSD) and systemic onset juvenile idiopathic arthritis (sJIA) and its use as a biomarker for diagnosis and evaluation of disease activity[J]. BMC Rheumatol, 2019, 3: 4. doi: 10.1186/s41927-019-0053-z
[17]	Yasin S, Fall N, Brown RA, et al. IL-18 as a biomarker linking systemic juvenile idiopathic arthritis and macrophage activation syndrome[J]. Rheumatology (Oxford), 2020, 59(2): 361-366. doi: 10.1093/rheumatology/kez282 pmid: 31326996
[18]	Krei JM, Møller HJ, Larsen JB. The role of interleukin-18 in the diagnosis and monitoring of hemophagocytic lymphohistiocytosis/macrophage activation syndrome - a systematic review[J]. Clin Exp Immunol, 2021, 203(2): 174-182. doi: 10.1111/cei.13543 pmid: 33128796
[19]	Prencipe G, Bracaglia C, De Benedetti F. Interleukin-18 in pediatric rheumatic diseases[J]. Curr Opin Rheumatol, 2019, 31(5): 421-427. doi: 10.1097/BOR.0000000000000634 pmid: 31192813
[20]	Juge PA, Crestani B, Dieudé P. Recent advances in rheumatoid arthritis-associated interstitial lung disease[J]. Curr Opin Pulm Med, 2020, 26(5): 477-486.

项目	肺受累sJIA组(n=41)	无肺受累sJIA组(n=32)	统计量	P
男[n(%)]	21(51.2)	19(59.4)	χ²=0.47	0.494
年龄[M(P₂₅~P₇₅)]/岁	5.5(2.8~9.3)	5.6(3.3~8.6)	Z=0.65	0.513
合并MAS[n(%)]	28(68.3)	4(12.5)	χ²=32.09	<0.001
发热[n(%)]	41(100)	32(100)	-	-
皮疹[n(%)]	36(87.8)	26(81.3)	χ²=0.59	0.444
关节痛[n(%)]	9(22.0)	6(18.8)	χ²=0.11	0.741
关节肿胀[n(%)]	13(31.7)	6(18.8)	χ²=1.56	0.216
乏力[n(%)]	19(46.3)	2(6.3)	χ²=16.99	<0.001
胸闷[n(%)]	17(41.5)	2(6.3)	χ²=16.99	<0.001
呼吸浅快[n(%)]	25(61.0)	4(12.5)	χ²=22.62	<0.001
呼吸困难[n(%)]	3(7.3)	1(3.1)	χ²=0.60	0.442
肝脾肿大[n(%)]	7(17.1)	1(3.1)	χ²=3.67	0.060
浅表淋巴结肿大[n(%)]	11(26.8)	4(12.5)	χ²=2.27	0.136
多浆膜腔积液[n(%)]	3(7.3)	3(9.4)	χ²=0.10	0.755
心脏增大[n(%)]	2(4.9)	1(3.1)	χ²=0.14	0.713
心功能异常[n(%)]	0(0)	0(0)	-	-
烦躁[n(%)]	5(12.2)	1(3.1)	χ²=1.96	0.166
易激惹[n(%)]	3(7.3)	1(3.1)	χ²=0.60	0.442
抽搐[n(%)]	0(0.0)	1(3.1)	χ²=1.29	0.261

项目	肺受累sJIA组(n=41)	无肺受累sJIA组(n=32)	统计量	P
WBC[M(P₂₅~P₇₅)]×10⁹·L^-1	18.3(15.6~22.2)	19.0(11.1~21.8)	Z=0.47	0.640
中性粒细胞[M(P₂₅~P₇₅)]/%	75.0(67.0~80.0)	73.0(60.3~85.8)	Z=0.09	0.930
PLT[M(P₂₅~P₇₅)]×10⁹·L^-1	496.0(318.5~668.5)	512.5(383.0~642.3)	Z=0.22	0.822
Hb(x±s)/g·L^-1	100.6±15.7	100.7±14.9	t=0.00	0.979
SF[M(P₂₅~P₇₅)]/ng·mL^-1	1 428.0(723.7~3 213.5)	1 641.0(733.75~4 391.0)	Z=0.51	0.614
ESR[M(P₂₅~P₇₅)]/mm·h^-1	87.0(62.0~101.5)	87.5(32.3~103.5)	Z=0.22	0.822
CRP[M(P₂₅~P₇₅)]/mg·L^-1	92.2(60.0~169.0)	93.1(55.5~172.0)	Z=0.31	0.758
CK[M(P₂₅~P₇₅)]/U·L^-1	29.0(20.0~77.0)	33(20.3~95.3)	Z=0.07	0.948
CKMB[M(P₂₅~P₇₅)]/U·L^-1	14.0(9.0~37.0)	15.5(8.0~42.5)	Z=0.10	0.918
LDH[M(P₂₅~P₇₅)]/U·L^-1	437.0(322.3~714.8)	543.0(314.0~736.5)	Z=0.08	0.940
AST[M(P₂₅~P₇₅)]/U·L^-1	85.0(41.5~487.5)	65(29.3~270.3)	Z=0.88	0.379
ALT[M(P₂₅~P₇₅)]/U·L^-1	98.0(30.4~481.8)	64.5(19.8~397.0)	Z=0.61	0.544
Na⁺(x±s)/mmol·L^-1	136.6±5.4	134.4±6.3	t=0.38	0.792
ALB(x±s)/g·L^-1	33.5±6.3	32.3±6.1	t=0.65	0.424
BUN[M(P₂₅~P₇₅)]/mmol·L^-1	6.0(5.0~6.9)	5.7(3.6~6.6)	Z=0.20	0.845
Cr(x±s)/μmol·L^-1	41.4±10.4	41.6±13.2	t=0.01	0.937
TG[M(P₂₅~P₇₅)]/mmol·L^-1	1.1(0.8~2.1)	1.3(0.9~1.8)	Z=0.37	0.708
FIb[M(P₂₅~P₇₅)]/g·L^-1	3.7(2.6~5.9)	5.0(2.7~6.4)	Z=1.68	0.192
D二聚体[M(P₂₅~P₇₅)]/mg·L^-1	1.1(0.7~1.5)	1.3(0.9~1.8)	Z=1.24	0.214
IL-18[M(P₂₅~P₇₅)]/pg·mL^-1	46.4(36.9~61.1)	34.5(26.4~43.9)	Z=3.67	<0.001
IL-6[M(P₂₅~P₇₅)]/pg·mL^-1	134.4(48.1~136.9)	134.7(39.3~138.3)	Z=0.08	0.933
胸片阳性改变[n(%)]	22(53.7)	4(12.5)	χ²=13.28	<0.001
肺部高分辨CT阳性改变[n(%)]	34(82.9)	5(15.6)	χ²=32.72	<0.001

因素	偏回归系数	标准误	χ²值	P	OR(95%CI)
IL-18	0.23	0.09	7.71	0.006	1.27(1.07~1.49)
胸片阳性改变	2.55	1.07	5.67	0.017	12.83(1.56~104.96)
肺高分辨CT阳性改变	0.18	0.09	4.19	0.041	1.19(1.01~1.41)
胸闷	2.41	1.22	3.88	0.049	11.15(1.01~122.72)
乏力	3.05	1.28	5.67	0.017	21.08(1.71~258.85)
合并MAS	1.98	1.02	3.77	0.052	7.23(0.98~52.21)
常量	-12.87	4.11	9.37	0.002	-

Clinical characteristics and related factors of pulmonary involvements in patients with systemic juvenile idiopathic arthritis

RichHTML

PDF (PC)

Like

Knowledge

Abstract

Cite this article

share this article

Figures/Tables 3

References 20

Related Articles 15

Metrics

Comments

Recommended 0

[1]	ZHAO Min, TANG Jihong, XIAO Xiao, YANG Letian, XU Huan, WU Yinyin, FENG Juan. Acute lymphoblastic leukemia with chemotherapy-related cerebral lesion: clinical and imaging features [J]. Journal of Clinical Pediatrics, 2025, 43(1): 14-20.
[2]	WANG Yuejia, XIA Lei, CHENG Huiqing, LYU Rongji, FAN Kun, XU Falin. Clinical characteristics of extremely premature infants conceived by assisted reproductive technology [J]. Journal of Clinical Pediatrics, 2024, 42(7): 600-605.
[3]	ZHONG Xiaomei, REN Hong, ZHANG Jian, LI Biru, QIAN Juan, NING Botao, GAO Yijin, WANG Ying. Comparison of clinical features between hemophagocytic lymphohistiocytosis and sepsis [J]. Journal of Clinical Pediatrics, 2024, 42(6): 547-552.
[4]	CHEN Shicai, DUAN Lifen, SUN Ying, SHAO Juwei, LI Qiong, LUO Mingying, REN Junjun, ZHANG Yunqian. Correlation between cognitive function and electroclinical characteristics of benign childhood epilepsy with centrotemporal spikes [J]. Journal of Clinical Pediatrics, 2024, 42(3): 211-217.
[5]	LIU Ping, JIANG Chunya, WEI Lei, WANG Xiangli, JIN Yunfeng, QING Yujie, YANG Yani, LIU Cuihua. Clinical features and long-term prognosis of 25 patients with systemic juvenile idiopathic arthritis complicated with macrophage activation syndrome [J]. Journal of Clinical Pediatrics, 2024, 42(3): 218-223.
[6]	HUANG Shihao, YUAN Xiaojun. Analysis on clinical characteristics and treatment for Kasabach-Merritt phenomenon in 36 children with hemangioma [J]. Journal of Clinical Pediatrics, 2024, 42(11): 917-921.
[7]	HOU Ruolin, WU Jing, LI Ling. Pediatric autoimmune encephalitis with brain MRI showing meningeal thickening and enhancement [J]. Journal of Clinical Pediatrics, 2023, 41(9): 674-679.
[8]	WU Xiaoling, LYU Tiewei. Clinical analysis of idiopathic left ventricular tachycardia in children [J]. Journal of Clinical Pediatrics, 2023, 41(8): 599-603.
[9]	HUA Minmin, XIA Lei, HUO Wanying, ZHANG Yanhua, XU Falin. Analysis of clinical and prognostic factors of neonatal congenital chylothorax [J]. Journal of Clinical Pediatrics, 2023, 41(1): 25-29.
[10]	MIAO Min, WANG Caiying, PANG Lin. Analysis of clinical characteristics of measles in 757 children [J]. Journal of Clinical Pediatrics, 2022, 40(8): 591-596.
[11]	XU Min, ZHOU Wenjie, TANG Jihong, SHI Xiaoyan, ZHANG Bingbing, XIAO Xiao. Clinical analysis of 51 cases of acute lymphoblastic leukemia with seizures in children [J]. Journal of Clinical Pediatrics, 2022, 40(2): 118-122.
[12]	QIU Lingzhi, ZHANG Yayuan, MA Le, FAN Zhidan, HUANG Na, YU Haiguo. Clinical analysis of fifteen patients with systemic juvenile idiopathic arthritis associated lung disease [J]. Journal of Clinical Pediatrics, 2022, 40(10): 750-754.
[13]	QIU Ling, CAO Jie. Analysis of clinical characteristics of childhood Miller-Fisher syndrome [J]. Journal of Clinical Pediatrics, 2021, 39(8): 565-.
[14]	TONG Pei, LIU Yan. Infantile neuroaxonal dystrophy and PLA2G6 gene mutation analysis: a case report [J]. Journal of Clinical Pediatrics, 2021, 39(4): 301-.
[15]	SUN Jinshan, CHEN Chaoying. Clinical features and prognostic analysis of complicated urinary tract infection in children [J]. Journal of Clinical Pediatrics, 2021, 39(2): 102-.