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Case Reports
. 2018 Sep 21;2(3):yty075.
doi: 10.1093/ehjcr/yty075. eCollection 2018 Sep.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) masquerading as acute ST-elevation myocardial infarction with complete resolution after immunosuppressive therapy: a case report

Joshua T Chai  1 , Sam McGrath  1 , Begoña Lopez  2 , Rafal Dworakowski  1
Affiliations
Case Reports

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) masquerading as acute ST-elevation myocardial infarction with complete resolution after immunosuppressive therapy: a case report

Joshua T Chai et al. Eur Heart J Case Rep. .

Abstract

Background: Eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg-Strauss syndrome) is a rare autoimmune condition characterized by inflammation of small- and medium-sized blood vessels, which usually presents with systemic vasculitis preceded by airway allergic hypersensitivity.

Case summary: Here, we report a highly unusual case of acute ST-elevation myocardial infarction in a young and fit man with no cardiovascular risk factors. His emergency coronary angiography revealed disproportionately severe widespread coronary artery disease. We describe the diagnostic challenges with emphasis on meticulous history-taking (deep hyponasal voice, anosmia, and childhood asthma), supported by timely blood markers (peripheral eosinophilia and raised CRP), and multi-modal imaging (severe paranasal sinusitis on cranial magnetic resonance imaging and multiple lung infiltrates with small patches of ground-glass appearance on thoracic computed tomography), to reach a diagnosis of EGPA coronary vasculitis with particular reference to the American College of Rheumatology EGPA classification. Importantly, with prompt immunosuppression, his coronary lesions resolved completely without the need of any surgical or percutaneous revascularisation. He remained well and asymptomatic on maintenance immunosuppressants at 1 year follow-up.

Discussion: This case highlighted the rare but recognized involvement of the coronary arteries in systemic EGPA vasculitis, which can sometimes mimic atherosclerotic coronary disease and acute coronary syndrome.

Keywords: Case report; Churg–Strauss; STEMI; Vasculitis.

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Figures

Figure 1
Figure 1
Presenting electrocardiogram and coronary angiography. Electrocardiogram showing inferior ST-segment elevation myocardial infarction. Coronary angiography at the time of presentation (A) showed unusually severe widespread luminal stenosis throughout the coronary trees despite administration of 500 μg of intracoronary glyceryl trinitrate; repeat angiography (B) after 4 weeks of immunosuppression showed complete resolution—note the smooth contour of the vessel wall devoid of atheroma (Standard LAO view for RCA and PA/slight RAO caudal view for LCA projection—please refer to Supplementary material online, Video clips for more detailed angiography).
Figure 2
Figure 2
Multi-modal investigation to support eosinophilic granulomatosis with polyangiitis diagnosis. Cardiac magnetic resonance imaging (A) showing acute myocardial infarction in the apical anterior and anterolateral walls but no myocarditis; computed tomography thorax (B) showing multiple tiny pulmonary nodules and small patches of ground-glass appearance (arrows) in the lung bases; cardiac computed tomography (C) showing no coronary calcification with an Agatston score of zero; asterisk denotes aortic root with left arrow at the right coronary artery and right arrow at the left main coronary artery; head magnetic resonance imaging (D) showing complete opacification of the maxillary sinuses (arrow).
See this image and copyright information in PMC

References

    1. Ibanez B, James S, Agewall S, Antunes MJ, Bucciarelli-Ducci C, Bueno H, et al.2017 ESC Guidelines for the management of acute myocardial infarction in patients presenting with ST-segment elevation: the Task Force for the management of acute myocardial infarction in patients presenting with ST-segment elevation of the European Society of Cardiology (ESC). Eur Heart J 2018;39:119. - PubMed
    1. Levine GN, Bates ER, Blankenship JC, Bailey SR, Bittl JA, Cercek B, Chambers CE, Ellis SG, Guyton RA, Hollenberg SM, Khot UN, Lange RA, Mauri L, Mehran R, Moussa ID, Mukherjee D, Ting HH, O’Gara PT, Kushner FG, Ascheim DD, Brindis RG, Casey DE, Chung MK, de Lemos JA, Diercks DB, Fang JC, Franklin BA, Granger CB, Krumholz HM, Linderbaum JA, Morrow DA, Newby LK, Ornato JP, Ou N, Radford MJ, Tamis-Holland JE, Tommaso CL, Tracy CM, Woo YJ, Zhao DX.. 2015 ACC/AHA/SCAI focused update on primary percutaneous coronary intervention for patients with ST-elevation myocardial infarction: an update of the 2011 ACCF/AHA/SCAI guideline for percutaneous coronary intervention and the 2013 ACCF/AHA guideline for the management of ST-elevation myocardial infarction. J Am Coll Cardiol 2016;67:1235–1250. - PubMed
    1. Churg J, Strauss L.. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951;27:277–301. - PMC - PubMed
    1. Sinico RA, Bottero P.. Churg-Strauss angiitis. Best Pract Res Clin Rheumatol 2009;23:355–366. - PubMed
    1. Martin RM, Wilton LV, Mann RD.. Prevalence of Churg-Strauss syndrome, vasculitis, eosinophilia and associated conditions: retrospective analysis of 58 prescription-event monitoring cohort studies. Pharmacoepidemiol Drug Saf 1999;8:179–189. - PubMed

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