Risk of macrophage activation syndrome in patients with adult-onset Still's disease with skin involvement: A retrospective cohort study

被引：10

作者：

Ding, Yuwei ^{[1
]}

Tang, Shunli ^{[1
]}

Li, Sheng ^{[1
]}

Yang, Changyi ^{[1
]}

Liu, Taoming ^{[1
]}

Ying, Shuni ^{[1
]}

Zhu, Dingxian ^{[1
]}

Sun, Chuanyin ^{[2
]}

Chen, Weiqian ^{[2
]}

Shi, Yu ^{[3
]}

Fang, Hong ^{[1
]}

Qiao, Jianjun ^{[1
]}

机构：

[1] Zhejiang Univ, Affiliated Hosp 1, Dept Dermatol, Sch Med, 79 Qingchun Rd, Hangzhou 310003, Zhejiang, Peoples R China

[2] Zhejiang Univ, Affiliated Hosp 1, Dept Rheumatol, Sch Med, Hangzhou, Peoples R China

[3] Zhejiang Univ, Affiliated Hosp 1, Dept Infect Dis, Sch Med, Hangzhou, Peoples R China

来源：

JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY | 2021年 / 85卷 / 06期

基金：

中国国家自然科学基金;

关键词：

adult-onset Still's disease; macrophage activation syndrome; persistent pruritic eruptions; prognosis; REACTIVE HEMOPHAGOCYTIC SYNDROME; JUVENILE IDIOPATHIC ARTHRITIS; CLASSIFICATION; CRITERIA;

D O I：

10.1016/j.jaad.2021.02.005

中图分类号：

R75 [皮肤病学与性病学];

学科分类号：

100206 ;

摘要：

Background: Small case series and case reports indicated that atypical persistent pruritic eruptions (PPEs), another type of skin lesions seen in adult-onset Still's disease (AOSD), imply a worse prognosis than typical evanescent rashes. Objective: To investigate clinical characteristics and macrophage activation syndrome (MAS) occurrence in AOSD with PPEs. Methods: A retrospective cohort study analyzed 150 patients with AOSD with rashes at the First Affiliated Hospital of Zhejiang University from January 2013 to December 2019. Results: Patients with AOSD with PPEs had higher lactate dehydrogenase (492.00 U/L vs 382.00 U/L; P < .001) and ferritin (6944.10 ng/ml vs 4286.60 ng/ml; P = .033), and lower fibrinogen (5.05 g/L vs 5.77 g/ L; P = .014) than those with evanescent rashes. Patients with AOSD with PPEs had a higher incidence (17.4% vs 3.1%, P = .006) and cumulative event rate for MAS (P = .008) and tended to receive high-dose glucocorticoid (36% vs 20.3%; P = .036). Multivariate analysis indicated that PPEs (hazard ratio [HR], 5.519; 95% confidence interval [CI], 1.138-26.767; P = .034), aspartate aminotransferase of greater than 120 U/L (HR, 8.084; 95% CI, 1.728-37.826; P = .008), and splenomegaly (HR, 21.152; 95% CI, 2.263-197.711; P = .007) were independent risk factors for MAS. Limitations: Single-center, retrospective nature, small sample size. Conclusion: PPEs indicated increased severity and MAS occurrence versus evanescent rashes. PPEs, aspartate aminotransferase of greater than 120 U/L, and splenomegaly were risk factors for MAS in AOSD with skin involvement.

引用

页码：1503 / 1509

页数：7

共 29 条

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