Journal of Clinical Pediatrics >
Urticaria as the initial manifestation of systemic juvenile idiopathic arthritis: a clinical perspective
Received date: 2025-12-16
Accepted date: 2026-04-13
Online published: 2026-05-08
This article reports the case of an 8-year-and-6-month-old female who presented with recurrent generalized urticarial lesions and remittent high fever. Initially suspected as chronic urticaria, her condition showed poor response to conventional treatments including anti-infectives, antihistamines, and glucocorticoids. The disease progressed rapidly with multisystem involvement and a marked elevation in inflammatory markers. Through multidisciplinary collaboration and systematic evaluation, she was ultimately diagnosed with systemic juvenile idiopathic arthritis (SJIA) complicated by macrophage activation syndrome (MAS). Her condition was gradually controlled with multitarget immunosuppressive therapy, including high-dose glucocorticoid pulse therapy, cyclosporine, and ruxolitinib. Based on this case, this article focuses on the differential diagnosis, diagnostic approach, and early recognition strategies for diseases presenting initially with urticarial lesions, aiming to enhance pediatricians' ability in the diagnosis and management of such conditions, thereby enabling early screening, intervention, and improved prognosis for complex and critical cases.
ZHANG Qihao , REN Yunqing . Urticaria as the initial manifestation of systemic juvenile idiopathic arthritis: a clinical perspective[J]. Journal of Clinical Pediatrics, 2026 , 44(5) : 445 -452 . DOI: 10.12372/jcp.2026.25e1599
| [1] | Fautrel B, Mitrovic S, De Matteis A, et al. EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease[J]. Ann Rheum Dis, 2024, 83(12): 1614-1627. |
| [2] | 中华医学会儿科学分会风湿病学组, 中国医师协会风湿免疫科医师分会儿科学组, 海峡两岸医药卫生交流协会儿童风湿免疫专业委员会, 等. 全身型幼年特发性关节炎合并巨噬细胞活化综合征诊疗专家共识(2025版)[J]. 中华实用儿科临床杂志, 2025, 40(6): 406-413. |
| The Subsequently Group of Pediatric Rheumatology, Society of Pediatrics, Chinese Medical Association; Subsequently Group of Pediatrics, Rheumatology and Immunology Physicians Committee, Chinese Medical Doctor Association; Committee of Pediatric Rheumatology and Immunology, Cross-Straits Medicine Exchange Association; et al. Expert consensus on the diagnosis and treatment of systemic juvenile idiopathic arthritis complicated with macrophage activation syndrome (2025 edition)[J]. Zhonghua Shiyong Erke Linchuan Zazhi, 2025, 40(6): 406-413. | |
| [3] | Brodell LA, Beck LA. Differential diagnosis of chronic urticaria[J]. Ann Allergy Asthma Immunol, 2008, 100(3): 181-190. |
| [4] | Dreyfus DH. Differential diagnosis of chronic urticaria and angioedema based on molecular biology, pharmacology, and proteomics[J]. Immunol Allergy Clin North Am, 2017, 37(1): 201-215. |
| [5] | Ferrer M, Bartra J, Giménez-Arnau A, et al. Management of urticaria: not too complicated, not too simple[J]. Clin Exp Allergy, 2015, 45(4): 731-743. |
| [6] | Kudryavtseva AV, Neskorodova KA, Staubach P. Urticaria in children and adolescents: An updated review of the pathogenesis and management[J]. Pediatr Allergy Immunol, 2019, 30(1): 17-24. |
| [7] | Zhou Y, Wang W, Zhong L, et al. Clinical and genetic spectrum of 14 cases of NLRP3-associated autoinflammatory disease (NLRP3-AID) in China and a review of the literature[J]. Orphanet J Rare Dis, 2022, 17(1): 214. |
| [8] | Cosson C, Riou R, Patoli D, et al. Functional diversity of NLRP3 gain-of-function mutants associated with CAPS autoinflammation[J]. J Exp Med, 2024, 221(5): e20231200. |
| [9] | Yang C, Chen Z, Zheng Y, et al. Low U-neck distribution of persistent pruritic eruptions in adult-onset Still disease: a retrospective cohort study with 65 patients[J]. J Am Acad Dermatol, 2025, 93(6): 1551-1553. |
| [10] | Bonnekoh H, Munoz M, Kolkhir P. A 58-year-old woman with urticaria, fever, and joint pain[J]. Jama, 2025, 333(8): 715-716. |
| [11] | Gusdorf L, Lipsker D. Neutrophilic urticarial dermatosis: a review[J]. Ann Dermatol Venereol, 2018, 145(12): 735-740. |
| [12] | 刘哲彰. 急性感染性荨麻疹的临床分析及相关炎症分子的初步探索[D]. 南昌大学, 2023. |
| Liu Zhezhe. Clinical analysis of acute infectious urticaria and preliminary exploration of related inflammatory molecules[D]. Nanchang University, 2023. | |
| [13] | Gorelik M, Chung SA, Ardalan K, et al. 2021 American college of rheumatology/vasculitis foundation guideline for the management of Kawasaki disease[J]. AArthritis Rheumatol, 2022, 74(4): 538-548. |
| [14] | 黄玉娟, 李夙凌, 田园, 等. 中国儿童川崎病诊疗循证指南(2023年)[J]. 中国当代儿科杂志, 2023, 25(12): 1198-1210. |
| Huang YJ, Li SL, Tian Y, et al. Evidence-based guideline on diagnosis and treatment of Kawasaki disease in Chinese children (2023)[J]. Zhongguo Dangdai Erke Zazhi 2023, 25(12): 1198-1210. | |
| [15] | Fukazawa R, Kobayashi J, Ayusawa M, et al. JCS/JSCS 2020 guideline on diagnosis and management of cardiovascular sequelae in Kawasaki disease[J]. Circ J, 2020, 84(8): 1348-1407. |
| [16] | 鞠晨晖, 肖燕燕, 高路, 等. 2024年版《关于川崎病诊断与管理的最新进展: 美国心脏协会科学声明》解读[J]. 中国实用儿科杂志, 2025, 40(8): 645-651. |
| Ju CH, Xiao YY, Gao L, et al. Interpretation of the 2024 edition of "recent advances in the diagnosis and management of Kawasaki disease: a scientific statement from the American Heart Association"[J]. Zhonghua Shiyong Erke Zazhi, 2025, 40(8): 645-651. | |
| [17] | Ergun T. Urticarial vasculitis[J]. Curr Opin Rheumatol, 2025, 37(1): 45-50. |
| [18] | Kolkhir P, Grakhova M, Bonnekoh H, et al. Treatment of urticarial vasculitis: a systematic review[J]. J Allergy Clin Immunol, 2019, 143(2): 458-466. |
| [19] | Marzano AV, Maronese CA, Genovese G, et al. Urticarial vasculitis: Clinical and laboratory findings with a particular emphasis on differential diagnosis[J]. J Allergy Clin Immunol, 2022, 149(4): 1137-1149. |
| [20] | Nguyen E, Gabel CK, Yu J. Pediatric drug eruptions[J]. Clin Dermatol, 2020, 38(6): 629-640. |
| [21] | Agrawal A, Arif SH, Kumarasan K, et al. Sweet's syndrome: an update[J]. Curr Pediatr Rev, 2022, 18(4): 265-273. |
| [22] | Cantillo JF, Garcia E, Fernandez-Caldas E, et al. Mosquito allergy: immunological aspects and clinical managemen[J]. Mol Immunol, 2023, 164: 153-158. |
| [23] | Valent P, Akin C, Sperr WR, et al. New insights into the pathogenesis of mastocytosis: emerging concepts in diagnosis and therapy[J]. Annu Rev Pathol, 2023, 18: 361-386. |
| [24] | Gialama D, Bonnekoh H, Rothermel ND, et al. Differential diagnosis of chronic spontaneous urticaria[J]. J Allergy Clin Immunol Pract, 2025, 13(9): 2242-2250. |
| [25] | 中华医学会皮肤性病学分会, 中国医师协会皮肤科医师分会. 中国难治性慢性自发性荨麻疹诊治指南(2025版)[J]. 中华皮肤科杂志, 2025, 58(6): 485-496. |
| Chinese Society of Dermatology and Venereology, Chinese Medical Association; China Dermatologist Association. Guideline for the diagnosis and treatment of difficult-to-treat chronic spontaneous urticaria in China (2025 edition)[J]. Zhonghua Pifuke Zazhi, 2025, 58(6): 485-496. | |
| [26] | 罗晰文, 唐雪梅. 全身型幼年特发性关节炎发病机制及精准诊治进展[J]. 中国实用儿科杂志, 2023, 38(6): 450-455. |
| Luo XW, Tang XM. Advances in pathogenesis, precise diagnosis and treatment of systemic juvenile idiopathic arthritis[J]. Zhongguo Shiyong Erke Zazhi, 2023, 38(6): 450-455. | |
| [27] | 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]. Rheumatic Disease Clinics, 2021, 47(4): 585-606. |
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