Summary
Definition
History and exam
Key diagnostic factors
- upper and lower respiratory tract involvement
- renal involvement
- constitutional features
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
Risk factors
- genetic predisposition
- infection
- environmental exposures
- white ethnicity
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
Investigations to consider
- tissue biopsy
- pulmonary function testing
- bronchoscopy
- electromyography/nerve conduction studies
- upper airway endoscopy
- CT sinuses
Treatment algorithm
severe (life/organ-threatening) disease: remission induction
nonsevere (non-life/organ-threatening) disease: remission induction
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.
Differentials
- Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome)
- Microscopic polyangiitis (MPA)
- Classic polyarteritis nodosa (cPAN)
More DifferentialsGuidelines
- 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
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