Granulomatose eosinofílica com poliangiite (Síndrome de Churg-Strauss)
Resumen
Definición
Anamnesis y examen
Principales factores de diagnóstico
- história de asma, rinite alérgica ou sinusite
- fraqueza ou dormência focal
- obstrução ou secreção nasal, ou dor facial
- púrpura palpável e petéquias
- sibilo
- hemoptise
- nódulos cutâneos
Otros factores de diagnóstico
- uso de determinados medicamentos
- fadiga, artralgias, mialgias
- dispneia ou tosse
- dor abdominal
- deficits sensoriais ou motores
- taquipneia
- estertores
- edema periférico
- ortopneia
- refluxo hepatojugular
Factores de riesgo
- história de asma, rinite alérgica ou sinusite
- uso de determinados medicamentos
Pruebas diagnósticas
Primeras pruebas diagnósticas para solicitar
- Hemograma completo com diferencial
- anticorpos anticitoplasma de neutrófilo (ANCA) séricos
- proteína C-reativa sérica
- velocidade de hemossedimentação
- ureia e creatinina séricas
- urinálise
- teste de função pulmonar
- radiografia torácica
- ecocardiograma
Pruebas diagnósticas que deben considerarse
- citometria de fluxo de sangue periférico
- testes moleculares para mutação FIP1L1/PDGFR alfa
- imunoglobulina E (IgE) sérica
- IgE e IgG séricas específicas para Aspergillus fumigatus
- coprocultura para ovos e parasitas
- Sorologia para Toxocara
- tomografia computadorizada (TC) do tórax
- eletromiografia
- broncoscopia com lavagem broncoalveolar (LBA)
- biópsia do tecido afetado
- ultrassonografia de membro inferior
- Angiotomografia
- biópsia da medula óssea
- nível da tiopurina metiltransferase
- teste de vírus da imunodeficiência humana (HIV)
Pruebas emergentes
- ressonância nuclear magnética (RNM) cardíaca
- eotaxina-3
Algoritmo de tratamiento
GEPA não grave
GEPA grave
recidiva após remissão bem-sucedida
Colaboradores
Autores
Karina Keogh, MBBCh
Assistant Professor
Division of Pulmonary and Critical Care Medicine
Mayo Clinic
Rochester
MN
Divulgaciones
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.
Agradecimientos
Dr Karina Keogh would like to gratefully acknowledge Dr Garvan Kane, a previous contributor to this topic.
Divulgaciones
GK is an author of some references cited in this topic.
Revisores por pares
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
Divulgaciones
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
Divulgaciones
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
Divulgaciones
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
Divulgaciones
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
Divulgaciones
RAO declares that he has no competing interests.
Agradecimiento de los revisores por pares
Los temas de BMJ Best Practice se actualizan de forma continua de acuerdo con los desarrollos en la evidencia y en las guías. Los revisores por pares listados aquí han revisado el contenido al menos una vez durante la historia del tema.
Divulgaciones
Las afiliaciones y divulgaciones de los revisores por pares se refieren al momento de la revisión.
Referencias
Artículos principales
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 Resumen
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 Resumen
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 Resumen
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 Resumen
Artículos de referencia
Una lista completa de las fuentes a las que se hace referencia en este tema está disponible para los usuarios con acceso a todo BMJ Best Practice.
Diferenciales
- Granulomatose com poliangiite (GPA) (anteriormente conhecida como granulomatose de Wegener)
- Poliangiíte microscópica (PAM)
- Síndrome hipereosinofílica (SHE)
Más DiferencialesGuías de práctica clínica
- Guideline for the prevention and treatment of glucocorticoid-induced osteoporosis
- Recommendations for the management of ANCA-associated vasculitis
Más Guías de práctica clínicaFolletos para el paciente
Asma em adultos: perguntas a fazer ao seu médico
Asma em adultos: o que é?
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