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)
Risk factors
- extremidades etárias
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
Investigations to consider
- angiografia convencional ou angiografia por ressonância magnética (ARM)
- tomografia por emissão de pósitrons (PET)
Treatment algorithm
suspeita de arterite de células gigantes
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
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
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.
Differentials
- Endocardite infecciosa
- Síndromes de hipercoagulabilidade
- Lúpus eritematoso sistêmico (LES)
More DifferentialsGuidelines
- American College of Rheumatology guideline for vaccinations in patients with rheumatic and musculoskeletal diseases
- EULAR recommendations for the management of ANCA-associated vasculitis
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