Granulomatosis with polyangiitis

Last reviewed: 27 Aug 2023
Last updated: 09 Mar 2023



History and exam

Key diagnostic factors

  • upper and lower respiratory tract involvement
  • renal involvement
  • constitutional features
More key diagnostic factors

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
Other diagnostic factors

Risk factors

  • genetic predisposition
  • infection
  • environmental exposures
  • white ethnicity
More risk factors

Diagnostic investigations

1st investigations 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
More 1st investigations to order

Investigations to consider

  • tissue biopsy
  • pulmonary function testing
  • bronchoscopy
  • electromyography/nerve conduction studies
  • upper airway endoscopy
  • CT sinuses
More investigations to consider

Treatment algorithm


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

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


remission successfully induced

relapse following successful remission



Eamonn Molloy, MD, MS, FRCPI

Consultant Rheumatologist

Department of Rheumatology

St Vincent's University Hospital




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



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



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

    • 2022 American College of Rheumatology guideline for vaccinations in patients with rheumatic and musculoskeletal disease
    • 2022 ACR recommendations for the prevention and treatment of glucocorticoid-induced osteoporosis summary
    More Guidelines
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