Kounis syndrome: a narrative review

Figures & data

Figure 1: Timeline of Kounis syndrome.²¹

Table 1: Mediators released by activated human mast cells (clinically important ones italicised)

Preformed mediators

Histamine Tryptase Chymase Renin Endothelin Carboxipeptidase Heparin proteoglycan Chondroitin sulfate proteoglycan Cathepsin G

Newly generated lipid-derived mediators

Platelet activating factor Leucotriene (LT) B4, LTC4, LTD4, LTE4 Prostaglandin (PG) D2, PGE2 Thromboxanes

Cytokines and chemokines

Inflammatory cytokines (interleukin (IL)-4, IL-5, IL-6, IL-13, tumour necrosis factor-α) Chemokines (monocyte chemotactic protein-1, IL-8, RANTES^*) Hematopoietic factors (IL-3, granulocyte-macrophage colony-stimulating factor) Growth factors (vascular endothelial growth factor, platelet derived growth factor etc.)

* Regulated on activation, normal T cell expressed and secreted.

Table 2: Clinical variants of Kounis syndrome

Clinical variant	Pathogenesis
Type I: vasospastic allergic angina	Patients with normal or nearly normal coronary arteries without risk factors for coronary atherosclerosis in which acute release of inflammatory mediators causes coronary spasm; (1) either without a rise in cardiac enzymes or (2) coronary spasm leading to an acute myocardial infarction (raise in cardiac enzymes). This may be due to endothelial dysfunction or micro-vascular angina
Type II: coronary thrombosis leading to allergic myocardial infarction	Patients with underlying coronary atherosclerosis in which inflammatory mediators causes either coronary spasm (with normal cardiac enzymes) or coronary spasm and plaque erosion or rupture leading to an acute myocardial infarction
Type III: coronary artery drug-eluting stent thrombosis	Hypersensitivity reaction to one of the components of the stent causing thrombosis. Giemsa and haemotoxalin-eosin staining shows mast cells and eosinophils respectively in the aspirated stent thrombus

Table 3: Clinical severity scale of immediate hypersensitivity reactions⁴⁷

Grade	Clinical signs
I	Muco-cutaneous signs: erythema, urticaria ± angioedema
II	Moderate multi-visceral signs: muco-cutaneous signs ± hypotension ± tachycardia ± dyspnoea ± gastrointestinal disturbances
III	Life-threatening mono- or multi-visceral signs: cardiovascular collapse, tachycardia/bradycardia ± arrhythmias ± bronchospasm ± muco-cutaneous signs ± gastrointestinal disturbance
IV	Cardiac arrest

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