Granulomatosis with polyangiitis

Last reviewed: 2 Jun 2022
Last updated: 28 Jun 2018



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
  • neurological manifestations
  • signs or symptoms of thromboembolism
  • white ethnicity
  • gastrointestinal involvement
  • cardiac involvement
  • breast mass
  • lower GU tract involvement
  • endocrine involvement
  • isolated mass lesions/focal granuloma
Other diagnostic factors

Risk factors

  • genetic predisposition
  • infection
  • environmental exposures
More risk factors

Diagnostic investigations

1st investigations to order

  • urinalysis and microscopy
  • CT chest
  • anti-neutrophil cytoplasmic antibody (ANCA)
  • FBC and differential
  • serum creatinine
  • erythrocyte sedimentation rate (ESR)
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


life/organ-threatening disease

non-life/organ-threatening disease excluding isolated subglottic stenosis

isolated subglottic stenosis


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 participated in local advisory boards for Pfizer, Roche, BMS, MSD, and Novartis. He has received conference travel/accommodation expenses from Pfizer, Roche, Abbvie, and MSD. The organisation he works for has an unrestricted educational grant from Roche to support regional educational meetings.

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

    • Recommendations for the prevention and treatment of glucocorticoid-induced osteoporosis
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