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Adult-onset Still disease

最后审阅: 18 Aug 2025
最后更新: 14 Sep 2023

小结

定义

病史和体格检查

关键诊断因素

  • fever ≥102.2°F (≥39.0°C)
  • arthralgia
  • arthritis
  • salmon-colored maculopapular skin rash
完整详情

其他诊断因素

  • dermatographic urticaria
  • sore throat
  • myalgia
  • lymphadenopathy
  • pleuritis
  • splenomegaly
  • hepatomegaly
  • pericarditis
  • myocarditis
  • signs of macrophage activation syndrome (MAS)
完整详情

危险因素

  • female sex
  • young adult age
  • preceding infection (e.g., with cytomegalovirus, Epstein-Barr virus, rubella, Mycoplasma)
完整详情

诊断性检查

首要检查

  • CBC
  • renal panel
  • C-reactive protein (CRP)
  • erythrocyte sedimentation rate (ESR)
  • liver function tests
  • procalcitonin
  • blood cultures
  • chest x-ray
  • renal and liver ultrasound scan
  • echocardiogram
  • ECG
完整详情

需考虑的检查

  • cardiac enzymes
  • cardiac MRI
  • serum ferritin
  • glycosylated ferritin
  • further tests as part of full septic screen
  • further tests as part of autoimmune/rheumatologic screen
  • fluorodeoxyglucose (FDG)-positron emission tomography (PET) whole-body scan
  • whole-body CT scan
  • bone marrow biopsy
  • lymph node biopsy
  • empirical corticosteroids
  • autoinflammatory gene profiling
完整详情

新兴检查

  • cytokine profiles
  • serum S100A12; serum calprotectin (S100A8/S100A9 dimer)
  • HLA genotyping

治疗流程

急症处理

severe disease: with severe organ dysfunction with or without signs of macrophage activation syndrome

持续性治疗

mild or moderate disease: no severe organ dysfunction or signs of macrophage activation syndrome

撰稿人

作者

Sinisa Savic, MRCP, FRCPath, PhD

Associate Professor (Clinical)

Leeds Institute of Rheumatic and Musculoskeletal Medicine

Consultant

Department of Clinical Immunology and Allergy

St James’s University Hospital

Clinical Lead for Allergy and Immunology

The Leeds Teaching Hospitals NHS Trust

Leeds

UK

Disclosures

SS has received payment for consulting services from Novartis, Takeda, SOBI, BioCryst, CSL Behring, and Kalvista. Speaking/educational events Takeda, Novartis, SOBI, and SCL Behring. Research grants SOBI and CSL Behring.

Adam Al-Hakim, BMBS, MRCP, BMedSci

Specialty Registrar in Clinical Immunology and Allergy

St James’s University Hospital

The Leeds Teaching Hospitals NHS Trust

Leeds

UK

Disclosures

AA declares that he has no competing interests.

Peer reviewers

Sreelakshmi Panginikkod, MD, FACP, FACR

Assistant Professor in Rheumatology and Associate Program Director for Rheumatology

Tufts University School of Medicine

Boston

MA

Disclosures

SP declares that she has no competing interests.

James Galloway, MBChB, MSc, PhD, FRCP

Professor of Rheumatology

King’s College London

Honorary Consultant Rheumatologist

King’s College Hospital

London

UK

Declarações

JG declares that he has no competing interests.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

Referências

Nossas equipes internas de editoria e de evidências trabalham em conjunto com colaboradores internacionais especializados e pares revisores para garantir que forneçamos acesso às informações o mais clinicamente relevantes possível.

Principais artigos

Giacomelli R, Ruscitti P, Shoenfeld Y. A comprehensive review on adult onset Still's disease. J Autoimmun. 2018 Sep;93:24-36.Texto completo  Resumo

Vordenbäumen S, Feist E, Rech J, et al. Diagnosis and treatment of adult-onset Still's disease: a concise summary of the German society of rheumatology S2 guideline. Z Rheumatol. 2023 Feb;82(suppl 2):81-92.Texto completo  Resumo

Macovei LA, Burlui A, Bratoiu I, et al. Adult-onset Still's disease-a complex disease, a challenging treatment. Int J Mol Sci. 2022 Oct 24;23(21):12810.Texto completo  Resumo

Efthimiou P, Kontzias A, Hur P, et al. Adult-onset Still's disease in focus: clinical manifestations, diagnosis, treatment, and unmet needs in the era of targeted therapies. Semin Arthritis Rheum. 2021 Aug;51(4):858-74.Texto completo  Resumo

Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still's disease. J Rheumatol. 1992 Mar;19(3):424-30. Resumo

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.
  • Adult-onset Still disease images
  • Diagnósticos diferenciais

    • Sepsis
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    • Biliary infection
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  • Diretrizes

    • Diagnosis and treatment of adult-onset Still's disease: a concise summary of the German society of rheumatology S2 guideline
    • Evidence-based clinical practice guideline for adult Still’s disease
    Mais Diretrizes
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