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Granulomatosis with polyangiitis

Última revisão: 18 Aug 2025
Última atualização: 18 Sep 2024

Resumo

Definição

ანამნეზი და გასინჯვა

ძირითადი დიაგნოსტიკური ფაქტორები

  • upper and lower respiratory tract involvement
  • renal involvement
  • constitutional features
სრული ტექსტი

სხვა დიაგნოსტიკური ფაქტორები

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

რისკფაქტორები

  • genetic predisposition
  • infection
  • environmental exposures
  • white ethnicity
სრული ტექსტი

დიაგნოსტიკური კვლევები

1-ად შესაკვეთი გამოკვლევები

  • urinalysis and microscopy
  • CT chest
  • anti-neutrophil cytoplasmic antibody (ANCA)
  • FBC and differential
  • serum creatinine
  • C-reactive protein
  • erythrocyte sedimentation rate (ESR)
  • liver function tests
  • serum calcium
სრული ტექსტი

გასათვალისწინებელი კვლევები

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

მკურნალობის ალგორითმი

მწვავე

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

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

მიმდინარე

remission successfully induced

relapse following successful remission

კონტრიბუტორები

ავტორები

Eamonn Molloy, MD, MS, FRCPI

Consultant Rheumatologist

Department of Rheumatology

St Vincent's University Hospital

Dublin

Ireland

გაფრთხილება:

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.

რეცენზენტები

Curry L. Koening, MD, MS

Assistant Professor of Medicine

Rheumatology Division

University of Utah School of Medicine

Salt Lake City

UT

გაფრთხილება:

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

利益声明

JMVL 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.

参考文献

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.

关键文献

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 Care Res (Hoboken). 2021 Aug;73(8):1088-105.全文  摘要

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.全文  摘要

Stone JH, Merkel PA, Spiera R, et al; RAVE-ITN Research Group. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010 Jul 15;363(3):221-32.全文  摘要

参考文献

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
  • Granulomatosis with polyangiitis images
  • 鉴别诊断

    • Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome)
    • Microscopic polyangiitis (MPA)
    • Classic polyarteritis nodosa (cPAN)
    更多 鉴别诊断
  • 指南

    • 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
    更多 指南
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