Churg-Strauss syndrome masquerading as acute MI
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.

The present case report, published in the European Heart Journal Case Reports, describes 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.

The authors 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.

Learning points
• In the presence of unusual pattern of diffuse coronary artery disease, clinicians should be alerted to the possibility of non-atherosclerotic coronary stenosis, for example, from coronary vasculitis that can sometimes represent an index presentation of eosinophilic granulomatosis with polyangiitis (EGPA)/Churg–Strauss syndrome.

• Clinical acumen with meticulous history supported by timely blood markers and imaging of the paranasal sinuses and thorax often allows a diagnosis to be made with good sensitivity and specificity.

• Coronary vasculitis from EGPA has been shown to be highly responsive to immunosuppressive therapy; in our case showing complete angiographic resolution of diffuse multi-vessel severe coronary stenosis within a short time of immunosuppression.

Read in detail here:
Dr. G●●●●●●●n T●●●●●●●●●●●m and 6 others like this1 share