Last reviewed: November 2017
Last updated: December  2016

Summary

Definition

History and exam

Key diagnostic factors

  • hx of asthma, allergic rhinitis, or sinusitis
  • focal numbness or weakness
  • nasal discharge or stuffiness, or facial pain
  • palpable purpura and petechiae
  • wheeze
  • haemoptysis
  • skin nodules

Other diagnostic factors

  • use of certain medications
  • fatigue, arthralgias, myalgias
  • shortness of breath or cough
  • abdominal pain
  • sensory or motor deficits
  • tachypnoea
  • rales
  • peripheral oedema
  • orthopnoea
  • hepatojugular reflux

Risk factors

  • hx of asthma, allergic rhinitis, or sinusitis
  • use of certain medications

Diagnostic investigations

1st investigations to order

  • FBC with differential
  • serum anti-neutrophil cytoplasmic antibodies (ANCA)
  • serum CRP
  • ESR
  • serum urea and creatinine
  • urinalysis
  • PFT
  • CXR
  • echocardiogram
Full details

Investigations to consider

  • flow cytometry of peripheral blood
  • molecular testing for FIP1L1/PDGFR alpha mutation
  • serum IgE
  • serum-specific IgE and IgG to Aspergillus fumigatus
  • stool culture for ova and parasites
  • Toxocara serology
  • CT chest
  • EMG
  • bronchoscopy with bronchoalveolar lavage (BAL)
  • biopsy of affected tissue
  • lower extremity ultrasound
  • CT angiography
  • bone marrow biopsy
  • thiopurine methyltransferase level
  • HIV testing
Full details

Emerging tests

  • cardiac MRI
  • eotaxin-3
Full details

Treatment algorithm

Contributors

Authors VIEW ALL

Assistant Professor

Division of Pulmonary and Critical Care Medicine

Mayo Clinic

Rochester

MN

Disclosures

KK is an author of a number of references cited in this monograph and has developed educational material on Churg-Strauss syndrome for First Consult (Elsevier). KK has undertaken contractual research for GlaxoSmithKline (utilizing mepolizumab and sirukumab in asthma trials), but received no personal financial gain for this work.

Dr Karina Keogh would like to gratefully acknowledge Dr Garvan Kane, a previous contributor to this monograph. GK is an author of a number of references cited in this monograph.

Peer reviewers VIEW ALL

Professor of Rheumatology

NIHR Musculoskeletal Biomedical Research Unit

Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Science

University of Oxford

Oxford

UK

Disclosures

RL has organisational financial interests with funds received for consultation and training on disease assessment in vasculitis for GlaxoSmithKline, Nordic, and Chemocentryx. RL has received personal funds for lectures on vasculitis from Roche and Nippon Kayaku. RL is an author of a reference cited in this monograph.

Clinical Senior Lecturer in Renal Medicine

Centre for Translational Inflammation Research

University of Birmingham Research Laboratories

Queen Elizabeth Hospital Birmingham

Birmingham

UK

Disclosures

MDM has been a paid consultant for Sphere Medical Ltd, and has received research grants and funding from GlaxoSmithKline and Novartis.

Assistant Professor of Medicine

Division of Rheumatology

Johns Hopkins University School of Medicine

Co-Director

Johns Hopkins Vasculitis Center

Baltimore

MD

Disclosures

PS declares that he has no competing interests.

Professor of Clinical Rheumatology

Institute of Cellular Medicine

Newcastle University

Newcastle upon Tyne

Honorary Consultant Rheumatologist

The James Cook University Hospital

Middlesbrough

UK

Disclosures

JVL is consultant for Roche, the manufacturer of rituximab, and has received speaker fees and reimbursements for attending several conferences.

Associate Professor and Director

Division of Rheumatology

University of Arkansas for Medical Sciences

Little Rock

AR

Disclosures

RAO declares that he has no competing interests.

Use of this content is subject to our disclaimer