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Granulomatosis with polyangiitis

Última revisão: 26 Mar 2025
Última atualização: 18 Sep 2024

Resumo

Definição

História e exame físico

Principais fatores diagnósticos

  • upper and lower respiratory tract involvement
  • renal involvement
  • constitutional features
Detalhes completos

Outros fatores diagnósticos

  • ocular manifestations
  • cutaneous manifestations
  • musculoskeletal manifestations
  • neurologic manifestations
  • signs or symptoms of thromboembolism
  • gastrointestinal involvement
  • cardiac involvement
  • breast mass
  • lower genitourinary tract involvement
  • endocrine involvement
  • isolated mass lesions/focal granuloma
Detalhes completos

Fatores de risco

  • genetic predisposition
  • infection
  • environmental exposures
  • white ethnicity
Detalhes completos

Investigações diagnósticas

Primeiras investigações a serem solicitadas

  • urinalysis and microscopy
  • CT chest
  • antineutrophil cytoplasmic antibody (ANCA)
  • CBC and differential
  • serum creatinine
  • C-reactive protein
  • erythrocyte sedimentation rate (ESR)
  • liver function tests
  • serum calcium
Detalhes completos

Investigações a serem consideradas

  • tissue biopsy
  • pulmonary function testing
  • bronchoscopy
  • electromyography/nerve conduction studies
  • upper airway endoscopy
  • CT sinuses
Detalhes completos

Algoritmo de tratamento

AGUDA

severe (life/organ-threatening) disease: remission induction

nonsevere (non-life/organ-threatening) disease: remission induction

CONTÍNUA

remission successfully induced

relapse following successful remission

Colaboradores

Autores

Eamonn Molloy, MD, MS, FRCPI

Consultant Rheumatologist

Department of Rheumatology

St Vincent's University Hospital

Dublin

Ireland

Declarações

EM declares that he has been a speaker for, and participated in advisory board for Abbvie, Janssen, Gilead, Novartis. He has received sponsorship to attend conferences from Abbvie, Janssen and UCB, and received research funding from Abbvie.

Revisores

Curry L. Koening, MD, MS

Assistant Professor of Medicine

Rheumatology Division

University of Utah School of Medicine

Salt Lake City

UT

Declarações

CLK declares that he has no competing interests.

Jaap M. van Laar, MD, PhD

Professor of Clinical Rheumatology

Musculoskeletal Research Group

Institute of Cellular Medicine

Newcastle University

The Medical School

Newcastle upon Tyne

UK

Declarações

JMVL declares that he has no competing interests.

  • Granulomatosis with polyangiitis images
  • Diagnósticos diferenciais

    • Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome)
    • Microscopic polyangiitis (MPA)
    • Classic polyarteritis nodosa (cPAN)
    Mais Diagnósticos diferenciais
  • Guidelines

    • KDIGO 2024 clinical practice guideline for the management of antineutrophil cytoplasmic antibody (ANCA) associated vasculitis
    • Guideline for vaccinations in patients with rheumatic and musculoskeletal disease
    Mais Guidelines
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