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Vasculite sistêmica

Última revisão: 1 May 2025
Última atualização: 29 Nov 2023

Summary

Definition

History and exam

Key diagnostic factors

  • 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)
Full details

Risk factors

  • extremidades etárias
Full details

Diagnostic investigations

1st investigations to order

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

Investigations to consider

  • angiografia convencional ou angiografia por ressonância magnética (ARM)
  • tomografia por emissão de pósitrons (PET)
Full details

Treatment algorithm

INITIAL

suspeita de arterite de células gigantes

ACUTE

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

ONGOING

resistente ao tratamento em qualquer estágio da terapia

Contributors

Authors

Brendan Antiochos, MD

Assistant Professor of Medicine

Director, Vasculitis Center

Division of Rheumatology

Johns Hopkins

Baltimore

MD

Disclosures

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

Acknowledgements

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

Disclosures

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

Peer reviewers

Jason M. Springer, MD, MS

Associate Professor

Vanderbilt University Medical Center

Nashville

TN

Disclosures

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

References

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.

Key articles

Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med. 1997 Nov 20;337(21):1512-23. Abstract

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. Abstract

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.Full text  Abstract

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.Full text  Abstract

Reference articles

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

    • Endocardite infecciosa
    • Síndromes de hipercoagulabilidade
    • Lúpus eritematoso sistêmico (LES)
    More Differentials
  • Guidelines

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