Skip to main content

Main menu

  • Home
  • Our journals
    • Clinical Medicine
    • Future Healthcare Journal
  • Subject collections
  • About the RCP
  • Contact us

Clinical Medicine Journal

  • ClinMed Home
  • Content
    • Current
    • Ahead of print
    • Archive
  • Author guidance
    • Instructions for authors
    • Submit online
  • About ClinMed
    • Scope
    • Editorial board
    • Policies
    • Information for reviewers
    • Advertising

User menu

  • Log in

Search

  • Advanced search
RCP Journals
Home
  • Log in
  • Home
  • Our journals
    • Clinical Medicine
    • Future Healthcare Journal
  • Subject collections
  • About the RCP
  • Contact us
Advanced

Clinical Medicine Journal

clinmedicine Logo
  • ClinMed Home
  • Content
    • Current
    • Ahead of print
    • Archive
  • Author guidance
    • Instructions for authors
    • Submit online
  • About ClinMed
    • Scope
    • Editorial board
    • Policies
    • Information for reviewers
    • Advertising

Eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome) masquerading as acute coronary syndrome

Peter Moore and Lin Thiri Toon
Download PDF
DOI: https://doi.org/10.7861/clinmed.20-2-s51
Clin Med March 2020
Peter Moore
AIsle of Wight NHS Trust, Newport, UK
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Lin Thiri Toon
AIsle of Wight NHS Trust, Newport, UK
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Info & Metrics
Loading

Case presentation

A 74-year-old man with a history of ankylosing spondylitis presented with a 5-day history of feeling generally unwell. He complained of burning retrosternal chest pain, cough productive of white sputum, shortness of breath, confusion, fevers and fatigue. He also mentioned seeing transient flashing lights. On examination, he had bibasal crackles on auscultation and splinter haemorrhages on his fingernails and toenails. Investigations revealed raised inflammatory markers, a raised white cell count (notably with a high eosinophilia), a raised troponin and D-dimer. Electrocardiography (ECG) showed incomplete right bundle branch block with left axis deviation and a chest X-ray was normal.

Initially, the patient was treated according to the acute coronary syndrome (ACS) protocol and was transferred to the coronary care unit. Following admission, he was investigated for infective endocarditis, but serial blood cultures were negative and echocardiography found no vegetations. Further investigations excluded parasitic infections and autoimmune conditions. The patient later developed slurred speech and decreased coordination. Computed tomography (CT) of his head showed a small low attenuation area in the right superior frontal lobe, reported as likely recently established ischaemic infarcts. CT of his chest/abdomen/pelvis excluded localised infections and occult malignancy, but showed a small pericardial effusion, pre-existing ankylosing spondylitis and bibasal pulmonary collapse/consolidation. Autoimmune screening showed a weak/moderately positive result for perinuclear anti-neutrophil cytoplasmic antibodies (pANCA) and a raised IgG. The diagnosis was eosinophilic granulomatosis with polyangiitis (EGPA) and the patient was treated with pulsed doses of intravenous methylprednisolone followed by oral prednisolone.

Background

EGPA (Churg–Strauss syndrome) is a rare autoimmune anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. According to the Revised International Chapel Hill consensus conference nomenclature of vasculitides (2012), it is defined as an eosinophil-rich and necrotising granulomatous inflammation often involving the respiratory tract, and necrotising vasculitis predominantly affecting small- to medium-sized vessels, associated with asthma and eosinophilia.1,2 The condition is a multisystem disorder and therefore often has non-specific presentations and multi-organ involvement. Typically, it has three stages of symptoms: the allergic stage, the eosinophilic stage and the vasculitic stage. These phases do not always occur sequentially and individuals may not develop all three phases. Phases last from 6 months to two years.3 There are few described cases of EGPA with cardiovascular presentations; manifesting as an ACS, acute myocarditis and cardiogenic shock, isolated cardiac tamponade, eosinophilic endomyocarditis with rapidly progressive diastolic dysfunction, the formation of apical thrombosis of both ventricles and ventricular tachycardia.4 The Lanham criteria and the American College of Rheumatology criteria are used for diagnosis, but blood tests are diagnostically useful. pANCA is positive in 40–60% of cases, eosinophilia is common but transient and elevation of IgG4 is also common.5,6 Treatment involves corticosteroids and cyclophosphamide (for remission induction), azathioprine and methotrexate (for remission maintenance) and B-cell depletion with rituximab has shown promising results for remission induction.1

Conclusion

EGPA is a rare autoimmune condition with non-specific presentations and multi-organ involvement. Eosinophilia, raised inflammatory markers and raised pANCA can be the clues for diagnosis, but raised troponin and D-dimer tests are sometimes misleading.

Conflicts of interest

None declared.

  • © Royal College of Physicians 2020. All rights reserved.

References

  1. ↵
    1. Jennette JC
    . Overview of the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. ClinExp Nephrol 2013;17:603–6.
    OpenUrl
  2. ↵
    1. Gioffredi A
    , Maritati F, Oliva E, Buzio C. Eosinophilic granulomatosis with polyangiitis: an overview. Front Immunol 2014;5:549.
    OpenUrlPubMed
  3. ↵
    1. National Organization for Rare Disorders.
    Churg Strauss syndrome. NORD, 2019. https://rarediseases.org/rare-diseases/churg-strauss-syndrome [Accessed 31 October 2019].
  4. ↵
    1. Budanova M
    , Mitrofanova L, Kozlenok A, et al. Ventricular tachycardia as the first manifestation of Churg–Strauss syndrome. J Cardiol Cases 2017;15:61–4.
    OpenUrl
  5. ↵
    1. Furuta S
    , Iwamoto T, Nakajima H. Update on eosinophilic granulomatosis with polyangiitis. Allergol Int 2019;68:430–6.
    OpenUrl
  6. ↵
    1. Science.gov
    . Sample records for background Churg-Strauss syndrome. Science.gov, 2019. www.science.gov/topicpages/b/background+churg-strauss+syndrome [Accessed 31 October 2019].
Back to top
Previous articleNext article

Article Tools

Download PDF
Article Alerts
Sign In to Email Alerts with your Email Address
Citation Tools
Eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome) masquerading as acute coronary syndrome
Peter Moore, Lin Thiri Toon
Clinical Medicine Mar 2020, 20 (Suppl 2) s51; DOI: 10.7861/clinmed.20-2-s51

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
Share
Eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome) masquerading as acute coronary syndrome
Peter Moore, Lin Thiri Toon
Clinical Medicine Mar 2020, 20 (Suppl 2) s51; DOI: 10.7861/clinmed.20-2-s51
del.icio.us logo Digg logo Reddit logo Twitter logo CiteULike logo Facebook logo Google logo Mendeley logo
  • Tweet Widget
  • Facebook Like
  • Google Plus One

Jump to section

  • Article
    • Case presentation
    • Background
    • Conclusion
    • Conflicts of interest
    • References
  • Info & Metrics

Related Articles

  • No related articles found.
  • PubMed
  • Google Scholar

Cited By...

  • No citing articles found.
  • Google Scholar

More in this TOC Section

  • Haemopoietic stem cell transplantation is a curative treatment option with minimal transplant-related complications for patients with severe Glanzmann's thrombasthenia
  • The monitoring and incidence of hyperglycaemia in inflammatory bowel disease patients treated with intravenous steroids
  • Clinical dynamics of nephropathy in patients with diabetes mellitus type 2 and concomitant essential hypertensive disease
Show more Clinical

Similar Articles

Navigate this Journal

  • Journal Home
  • Current Issue
  • Ahead of Print
  • Archive

Related Links

  • ClinMed - Home
  • FHJ - Home
clinmedicine Footer Logo
  • Home
  • Journals
  • Contact us
  • Advertise
HighWire Press, Inc.

Follow Us:

  • Follow HighWire Origins on Twitter
  • Visit HighWire Origins on Facebook

Copyright © 2021 by the Royal College of Physicians