Clinical features of Kawasaki disease complicated by macrophage activation syndrome: an analysis of 27 cases

WEN Yi-Ni, CHEN Jing, LIU Fan, DING Yan, YIN Wei

Chinese Journal of Contemporary Pediatrics ›› 2023, Vol. 25 ›› Issue (6) : 572-578.

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Chinese Journal of Contemporary Pediatrics ›› 2023, Vol. 25 ›› Issue (6) : 572-578. DOI: 10.7499/j.issn.1008-8830.2302015
TOPIC OF KAWASAKI DISEASE

Clinical features of Kawasaki disease complicated by macrophage activation syndrome: an analysis of 27 cases

  • WEN Yi-Ni, CHEN Jing, LIU Fan, DING Yan, YIN Wei
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Abstract

Objective To investigate the clinical manifestations and laboratory examination results of children with Kawasaki disease complicated by macrophage activation syndrome (KD-MAS), and to provide a basis for identifying early warning indicators for the early diagnosis and treatment of KD-MAS. Methods A retrospective study was performed on 27 children with KD-MAS (KD-MAS group) and 110 children with KD (KD group) who were admitted to Wuhan Children's Hospital, Tongji Medical College, Huazhong University of Science and Technology, from January 2014 to January 2022. Clinical and laboratory data were compared between the two groups. The receiver operating characteristic (ROC) curve was used to investigate the value of laboratory markers with statistical significance in the diagnosis of KD-MAS. Results Compared with the KD group, the KD-MAS group had significantly higher incidence rates of hepatomegaly, splenomegaly, incomplete KD, no response to intravenous immunoglobulin, coronary artery damage, multiple organ damage, and KD recurrence, as well as a significantly longer length of hospital stay (P<0.05). Compared with the KD group, the KD-MAS group had significantly lower levels of white blood cell count, absolute neutrophil count, hemoglobin, platelet count (PLT), erythrocyte sedimentation rate, serum albumin, serum sodium, prealbumin, and fibrinogen (FIB), a significantly lower incidence rate of non-exudative conjunctiva, and significantly higher levels of C-reactive protein, alanine aminotransferase, aspartate aminotransferase, lactate dehydrogenase (LDH), and serum ferritin (SF) (P<0.05). The ROC curve analysis showed that SF, PLT, FIB, and LDH had high value in the diagnosis of KD-MAS, with areas under the curve (AUC) of 0.989, 0.966, 0.932, and 0.897, respectively (P<0.001), and optimal cut-off values of 349.95 μg/L, 159×109/L, 3.85 g/L, and 403.50 U/L, respectively. The combination of SF, PLT, FIB, and LDH had a larger AUC than PLT, FIB, and LDH alone in the diagnosis of KD-MAS (P<0.05), but there was no significant difference in the AUC between the combination of SF, PLT, FIB, and LDH and SF alone (P>0.05). Conclusions KD-MAS should be considered when children with KD have hepatosplenomegaly, no response to intravenous immunoglobulin, coronary artery damage, and KD recurrence during treatment. SF, PLT, FIB, and LDH are of high value in the diagnosis of KD-MAS, especially SF is of great significance in the diagnosis of KD-MAS.

Key words

Kawasaki disease / Macrophage activation syndrome / Clinical feature / Child

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WEN Yi-Ni, CHEN Jing, LIU Fan, DING Yan, YIN Wei. Clinical features of Kawasaki disease complicated by macrophage activation syndrome: an analysis of 27 cases[J]. Chinese Journal of Contemporary Pediatrics. 2023, 25(6): 572-578 https://doi.org/10.7499/j.issn.1008-8830.2302015

References

1 Newburger JW, Takahashi M, Gerber MA, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association[J]. Circulation, 2004, 110(17): 2747-2771. PMID: 15505111. DOI: 10.1161/01.CIR.0000145143.19711.78.
2 Alongi A, Naddei R, De Miglio L, et al. Macrophage activation syndrome in pediatrics[J]. Pediatr Allergy Immunol, 2020, 31 Suppl 24: 13-15. PMID: 32017214. DOI: 10.1111/pai.13158.
3 Sen ES, Clarke SL, Ramanan AV. Macrophage activation syndrome[J]. Indian J Pediatr, 2016, 83(3): 248-253. PMID: 26400031. DOI: 10.1007/s12098-015-1877-1.
4 McCrindle BW, Rowley AH, Newburger JW, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association[J]. Circulation, 2017, 135(17): e927-e999. PMID: 28356445. DOI: 10.1161/CIR.0000000000000484.
5 Ravelli A, Minoia F, Davì S, et al. 2016 classification criteria for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation collaborative initiative[J]. Ann Rheum Dis, 2016, 75(3): 481-489. PMID: 26865703. DOI: 10.1136/annrheumdis-2015-208982.
6 Islam MI, Talukder MK, Islam MM, et al. Macrophage activation syndrome in paediatric rheumatic diseases[J]. Mymensingh Med J, 2017, 26(2): 356-363. PMID: 28588173.
7 何涛, 钟家蓉, 张静. 川崎病并发巨噬细胞活化综合征9例病例报告并文献复习[J]. 中国循证儿科杂志, 2018, 13(6): 421-426. DOI: 10.3969/j.issn.1673-5501.2018.06.005.
8 何胜男, 唐雪梅, 张宇, 等. 川崎病相关巨噬细胞活化综合征临床分析及诊断标准初探[J]. 中华实用儿科临床杂志, 2018, 33(9): 679-683. DOI: 10.3760/cma.j.issn.2095-428X.2018.09.007.
9 García-Pavón S, Yamazaki-Nakashimada MA, Báez M, et al. Kawasaki disease complicated with macrophage activation syndrome: a systematic review[J]. J Pediatr Hematol Oncol, 2017, 39(6): 445-451. PMID: 28562511. DOI: 10.1097/MPH.0000000000000872.
10 Jinkawa A, Shimizu M, Nishida K, et al. Cytokine profile of macrophage activation syndrome associated with Kawasaki disease[J]. Cytokine, 2019, 119: 52-56. PMID: 30877950. DOI: 10.1016/j.cyto.2019.03.001.
11 Choi JE, Kwak Y, Huh JW, et al. Differentiation between incomplete Kawasaki disease and secondary hemophagocytic lymphohistiocytosis following Kawasaki disease using N-terminal pro-brain natriuretic peptide[J]. Korean J Pediatr, 2018, 61(5): 167-173. PMID: 29853942. PMCID: PMC5976567. DOI: 10.3345/kjp.2018.61.5.167.
12 Wang W, Gong F, Zhu W, et al. Macrophage activation syndrome in Kawasaki disease: more common than we thought?[J]. Semin Arthritis Rheum, 2015, 44(4): 405-410. PMID: 25200945. DOI: 10.1016/j.semarthrit.2014.07.007.
13 Pilania RK, Jindal AK, Johnson N, et al. Macrophage activation syndrome in children with Kawasaki disease: an experience from a tertiary care hospital in northwest India[J]. Rheumatology (Oxford), 2021, 60(7): 3413-3419. PMID: 33221920. DOI: 10.1093/rheumatology/keaa715.
14 Jin P, Luo Y, Liu X, et al. Kawasaki disease complicated with macrophage activation syndrome: case reports and literature review[J]. Front Pediatr, 2019, 7: 423. PMID: 31737585. PMCID: PMC6838014. DOI: 10.3389/fped.2019.00423.
15 Minoia F, Davì S, Horne A, et al. Clinical features, treatment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a multinational, multicenter study of 362 patients[J]. Arthritis Rheumatol, 2014, 66(11): 3160-3169. PMID: 25077692. DOI: 10.1002/art.38802.
16 中国医师协会儿科医师分会风湿免疫学组, 中国儿童免疫与健康联盟, 《中国实用儿科杂志》编辑委员会. 儿童风湿性疾病相关巨噬细胞活化综合征诊断与治疗专家共识之五——川崎病篇[J]. 中国实用儿科杂志, 2020, 35(11): 841-845. DOI: 10.19538/j.ek2020110605.
17 汪顺雨, 刘庚英, 杜忠东. 川崎病合并巨噬细胞活化综合征的临床研究进展[J]. 中华儿科杂志, 2020, 58(11): 953-956. PMID: 33120474. DOI: 10.3760/cma.j.cn112140-20200407-00354.
18 Roh DE, Kwon JE, Choi HJ, et al. Importance of serum ferritin level for early diagnosis and differentiation in patients with Kawasaki disease with macrophage activation syndrome[J]. Children (Basel), 2021, 8(4): 269. PMID: 33807266. PMCID: PMC8065514. DOI: 10.3390/children8040269.
19 Cron RQ, Davi S, Minoia F, et al. Clinical features and correct diagnosis of macrophage activation syndrome[J]. Expert Rev Clin Immunol, 2015, 11(9): 1043-1053. PMID: 26082353. DOI: 10.1586/1744666X.2015.1058159.
20 Lind-Holst M, Hartling UB, Christensen AE. High-dose anakinra as treatment for macrophage activation syndrome caused by refractory Kawasaki disease in an infant[J]. BMJ Case Rep, 2019, 12(8): e229708. PMID: 31383678. PMCID: PMC6685371. DOI: 10.1136/bcr-2019-229708.
21 Pal P, Bathia J, Giri PP, et al. Macrophage activation syndrome in pediatrics: 10 years data from an Indian center[J]. Int J Rheum Dis, 2020, 23(10): 1412-1416. PMID: 32666639. DOI: 10.1111/1756-185X.13915.
22 Lerkvaleekul B, Vilaiyuk S. Macrophage activation syndrome: early diagnosis is key[J]. Open Access Rheumatol, 2018, 10: 117-128. PMID: 30214327. PMCID: PMC6124446. DOI: 10.2147/OARRR.S151013.
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