Summary
Definição
História e exame físico
Principais fatores diagnósticos
- history of asthma, allergic rhinitis, or sinusitis
- focal numbness or weakness
- nasal discharge or stuffiness, or facial pain
- palpable purpura and petechiae
- wheeze
- hemoptysis
- skin nodules
Outros fatores diagnósticos
- use of certain medications
- fatigue, arthralgias, myalgias
- shortness of breath or cough
- abdominal pain
- sensory or motor deficits
- tachypnea
- rales
- peripheral edema
- orthopnea
- hepatojugular reflux
Fatores de risco
- history of asthma, allergic rhinitis, or sinusitis
- use of certain medications
Investigações diagnósticas
Primeiras investigações a serem solicitadas
- CBC with differential
- serum antineutrophil cytoplasmic antibodies (ANCA)
- serum CRP
- erythrocyte sedimentation rate
- serum BUN and creatinine
- urinalysis
- pulmonary function test
- chest x-ray
- echocardiography
Investigações a serem consideradas
- flow cytometry of peripheral blood
- molecular testing for FIP1L1/PDGFR alpha mutation
- serum IgE
- serum-specific IgE and IgG to Aspergillus fumigatus
- stool culture for ova and parasites
- Toxocara serology
- CT chest
- electromyogram
- bronchoscopy with bronchoalveolar lavage (BAL)
- biopsy of affected tissue
- lower extremity ultrasound
- CT angiography
- bone marrow biopsy
- thiopurine methyltransferase level
- HIV testing
Novos exames
- cardiac MRI
- eotaxin-3
Algoritmo de tratamento
nonsevere EGPA
severe EGPA
relapse following successful remission
Colaboradores
Autores
Karina Keogh, MBBCh
Assistant Professor
Division of Pulmonary and Critical Care Medicine
Mayo Clinic
Rochester
MN
Declarações
KK is an author of a number of references cited in this topic and has developed educational material on Churg-Strauss syndrome for First Consult (Elsevier), and received an education grant from GlaxoSmithKline for lectures given on EGPA at Boston University. KK has undertaken contractual research for GlaxoSmithKline (utilizing mepolizumab in asthma trials) and AstraZeneca (asthma trial), but received no personal financial gain for this work.
Agradecimentos
Dr Karina Keogh would like to gratefully acknowledge Dr Garvan Kane, a previous contributor to this topic.
Declarações
GK is an author of some references cited in this topic.
Revisores
Raashid Luqmani, DM, FRCP, FRCP(E)
Professor of Rheumatology
NIHR Musculoskeletal Biomedical Research Unit
Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Science
University of Oxford
Oxford
UK
Declarações
RL has organisational financial interests with funds received for consultation and training on disease assessment in vasculitis for GlaxoSmithKline, Nordic, and Chemocentryx. RL has received personal funds for lectures on vasculitis from Roche and Nippon Kayaku. RL is an author of a reference cited in this topic.
Matthew D. Morgan, MB ChB, MRCP, PhD
Clinical Senior Lecturer in Renal Medicine
Centre for Translational Inflammation Research
University of Birmingham Research Laboratories
Queen Elizabeth Hospital Birmingham
Birmingham
UK
Declarações
MDM has been a paid consultant for Sphere Medical Ltd, and has received research grants and funding from GlaxoSmithKline and Novartis.
Philip Seo, MD, MHS
Assistant Professor of Medicine
Division of Rheumatology
Johns Hopkins University School of Medicine
Co-Director
Johns Hopkins Vasculitis Center
Baltimore
MD
Declarações
PS declares that he has no competing interests.
Jaap Van Laar, MD, PhD
Professor of Clinical Rheumatology
Institute of Cellular Medicine
Newcastle University
Newcastle upon Tyne
Honorary Consultant Rheumatologist
The James Cook University Hospital
Middlesbrough
UK
Declarações
JVL is consultant for Roche, the manufacturer of rituximab, and has received speaker fees and reimbursements for attending several conferences.
Robert A. Ortmann, MD
Associate Professor and Director
Division of Rheumatology
University of Arkansas for Medical Sciences
Little Rock
AR
Declarações
RAO 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
Principais artigos
Ntatsaki E, Carruthers D, Chakravarty K, et al; BSR and BHPR Standards, Guidelines and Audit Working Group. BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis. Rheumatology (Oxford). 2014 Dec;53(12):2306-9.Texto completo Resumo
Groh M, Pagnoux C, Baldini C, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. Eur J Intern Med. 2015 Sep;26(7):545-53.Texto completo Resumo
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.Texto completo Resumo
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:ard-2022-223764.Texto completo Resumo
Artigos de referência
Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.
Diagnósticos diferenciais
- Granulomatosis with polyangiitis (GPA) (formerly known as Wegener granulomatosis)
- Microscopic polyangiitis (MPA)
- Hypereosinophilic syndrome (HES)
Mais Diagnósticos diferenciaisDiretrizes
- Guideline for the prevention and treatment of glucocorticoid-induced osteoporosis
- Recommendations for the management of ANCA-associated vasculitis
Mais DiretrizesFolhetos informativos para os pacientes
Asthma in adults
Asthma in adults: what are the treament options?
Mais Folhetos informativos para os pacientes
Conectar-se ou assinar para acessar todo o BMJ Best Practice
O uso deste conteúdo está sujeito ao nosso aviso legal