Granulomatosis with polyangiitis

Last reviewed: 8 Nov 2022
Last updated: 12 Jul 2022

Summary

Definition

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
  • 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
  • anti-neutrophil cytoplasmic antibody (ANCA)
  • FBC 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

ACUTE

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

non-severe (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 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

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

    • 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis
    • 2020 American College of Rheumatology guideline for the management of reproductive health in rheumatic and musculoskeletal diseases
    More Guidelines
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