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Granulomatosis with polyangiitis

Last reviewed: 10 Nov 2024
Last updated: 18 Sep 2024

Summary

Definition

History and exam

Key diagnostic factors

  • upper and lower respiratory tract involvement
  • renal involvement
  • constitutional features
Full details

Other diagnostic factors

  • 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
Full details

Risk factors

  • genetic predisposition
  • infection
  • environmental exposures
  • white ethnicity
Full details

Diagnostic tests

1st tests to order

  • 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
Full details

Tests to consider

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

Treatment algorithm

ACUTE

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

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

ONGOING

remission successfully induced

relapse following successful remission

Contributors

Authors

Eamonn Molloy, MD, MS, FRCPI

Consultant Rheumatologist

Department of Rheumatology

St Vincent's University Hospital

Dublin

Ireland

Disclosures

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.

Peer reviewers

Curry L. Koening, MD, MS

Assistant Professor of Medicine

Rheumatology Division

University of Utah School of Medicine

Salt Lake City

UT

Disclosures

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

Disclosures

JMVL declares that he has no competing interests.

  • Granulomatosis with polyangiitis images
  • Differentials

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