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Vasculite sistêmica

Última revisão: 20 Aug 2025
Última atualização: 29 Nov 2023

Resumo

Definição

病史和体格检查

关键诊断因素

  • presença de fatores de risco
  • sintomas constitucionais (por exemplo, mal-estar, febre, artralgia, mialgia)
  • cefaleia e sensibilidade no couro cabeludo (arterite de células gigantes)
  • alterações visuais (vasos grandes)
  • claudicação da mandíbula ou dos membros superiores (grandes vasos)
  • pulsos braquiais assimétricos (grandes vasos)
  • sopros (grandes vasos)
  • dor abdominal (vasos médios)
  • pé pendente, pulso pendente (vasos médios)
  • úlceras cutâneas (vasos médios)
  • hematúria (pequenos vasos)
  • púrpura palpável (pequenos vasos)
  • otorreia, dor ou sensação de abafamento nos ouvidos (pequenos vasos)
  • sintomas nasais (pequenos vasos)
  • dor sinusal (pequenos vasos)
  • sibilo (pequenos vasos)
  • hemoptise (pequenos vasos)
完整详情

危险因素

  • extremidades etárias
完整详情

诊断性检查

首要检查

  • velocidade de hemossedimentação (VHS)
  • proteína C-reativa
  • autoanticorpos anticitoplasma de neutrófilos (ANCA)
  • avaliação da glomerulonefrite
  • biópsia do tecido afetado
完整详情

需考虑的检查

  • angiografia convencional ou angiografia por ressonância magnética (ARM)
  • tomografia por emissão de pósitrons (PET)
完整详情

治疗流程

初步治疗

suspeita de arterite de células gigantes

急症处理

granulomatose com poliangiite (GPA) ou poliangiite microscópica (PAM) com risco de vida ou com risco para órgãos

granulomatose com poliangiite (GPA) ou poliangiite microscópica (PAM) sem risco de vida ou sem risco para órgãos

持续性治疗

resistente ao tratamento em qualquer estágio da terapia

撰稿人

作者

Brendan Antiochos, MD

Assistant Professor of Medicine

Director, Vasculitis Center

Division of Rheumatology

Johns Hopkins

Baltimore

MD

利益声明

BA has received compensation from Paradigm Medical Communications for CME activities regarding EGPA and Giant Cell Arteritis.

鸣谢

Dr Brendan Antiochos would like to gratefully acknowledge Dr Phillip Seo, the previous contributor for this topic.

利益声明

PS is an author of some references cited in this topic.

同行评议者

Jason M. Springer, MD, MS

Associate Professor

Vanderbilt University Medical Center

Nashville

TN

利益声明

JMS has served as a consultant and on an advisory board for ChemoCentryx, the manufacturer of avacopan.

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.

参考文献

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.

关键文献

Hellmich B, Agueda A, Monti S, et al. 2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2020 Jan;79(1):19-30. 摘要

Chung SA, Langford CA, Maz M, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol. 2021 Aug;73(8):1366-83.全文  摘要

Hellmich B, Sanchez-Alamo B, Schirmer JH, et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Ann Rheum Dis. 2023 Mar 16;2022-223764.全文  摘要

参考文献

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
  • Vasculite sistêmica images
  • 鉴别诊断

    • Endocardite infecciosa
    • Síndromes de hipercoagulabilidade
    • Lúpus eritematoso sistêmico (LES)
    更多 鉴别诊断
  • Guías de práctica clínica

    • American College of Rheumatology guideline for vaccinations in patients with rheumatic and musculoskeletal diseases
    • EULAR recommendations for the management of ANCA-associated vasculitis
    更多 指南
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