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Adult-onset Still disease

Последний просмотренный: 14 Aug 2025
Last updated: 14 Sep 2023

Резюме

Определение

Анамнез и осмотр

Ключевые диагностические факторы

  • fever ≥102.2°F (≥39.0°C)
  • arthralgia
  • arthritis
  • salmon-colored maculopapular skin rash
Полная информация

Другие диагностические факторы

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

Факторы риска

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

Диагностические исследования

Исследования, которые показаны в первую очередь

  • 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
Полная информация

Исследования, проведение которых нужно рассмотреть

  • 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
Полная информация

Неотложные исследования

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

Алгоритм лечения

Острый

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

ПРОДОЛЖЕНИЕ

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

Составители

Авторы

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

Раскрытие информации

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

Раскрытие информации

AA declares that he has no competing interests.

Рецензенты

Sreelakshmi Panginikkod, MD, FACP, FACR

Assistant Professor in Rheumatology and Associate Program Director for Rheumatology

Tufts University School of Medicine

Boston

MA

Раскрытие информации

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

Раскрытие информации

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.

Список литературы

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Основные статьи

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

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.Полный текст  Аннотация

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.Полный текст  Аннотация

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.Полный текст  Аннотация

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. Аннотация

Статьи, указанные как источники

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