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Risk of macrophage activation syndrome in patients with adult-onset Still's disease with skin involvement: A retrospective cohort study.
Ding, Yuwei; Tang, Shunli; Li, Sheng; Yang, Changyi; Liu, Taoming; Ying, Shuni; Zhu, Dingxian; Sun, Chuanyin; Chen, Weiqian; Shi, Yu; Fang, Hong; Qiao, Jianjun.
Afiliação
  • Ding Y; Department of Dermatology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.
  • Tang S; Department of Dermatology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.
  • Li S; Department of Dermatology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.
  • Yang C; Department of Dermatology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.
  • Liu T; Department of Dermatology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.
  • Ying S; Department of Dermatology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.
  • Zhu D; Department of Dermatology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.
  • Sun C; Department of Rheumatology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.
  • Chen W; Department of Rheumatology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.
  • Shi Y; Department of Infectious Diseases, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.
  • Fang H; Department of Dermatology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China. Electronic address: fanghongzy@zju.edu.cn.
  • Qiao J; Department of Dermatology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China. Electronic address: qiaojianjun@zju.edu.cn.
J Am Acad Dermatol ; 85(6): 1503-1509, 2021 12.
Article em En | MEDLINE | ID: mdl-33556412
ABSTRACT

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.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Doença de Still de Início Tardio / Exantema / Síndrome de Ativação Macrofágica Tipo de estudo: Diagnostic_studies / Etiology_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Limite: Adult / Humans Idioma: En Ano de publicação: 2021 Tipo de documento: Article

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Doença de Still de Início Tardio / Exantema / Síndrome de Ativação Macrofágica Tipo de estudo: Diagnostic_studies / Etiology_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Limite: Adult / Humans Idioma: En Ano de publicação: 2021 Tipo de documento: Article