Cardiac involvement in eosinophilic granulomatosis with polyangitis (Churg Strauss) (RCD code: I-3A.7a)

Wojciech Szczeklik, Tomasz Miszalski-Jamka

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Abstract


Eosinophilic granulomatosis with polyangitis – EGPA (previously known as Churg‑Strauss syndrome) is a  rare, systemic, necrotizing small‑vessel vasculitis with accompanying bronchial asthma, eosinophilia and eosinophilic tissue infiltration of various tissues with granuloma formation. The pathognomonic laboratory feature of EGPA is prominent peripheral eosinophilia that commonly exceed 1,500 cells/μl, and the presence in approximately 40% of patients of the antineutrophil autoantibodies (ANCA). EGPA is one of the most common of the systemic vasculitides to affect the heart and if present it deteriorates the prognosis of the disease and increases mortality. Cardiac involvement varies widely ranging from 16–92% and is more common in ANCA negative patients. The cardiac manifestations varies in its clinical presentation. It may present as myocarditis with cardiomyopathy, pericarditis with pericardial effusion (up to 25% of patients), heart failure (18%),varies ventricular and supraventricular arrhythmias, valve involvement and sudden cardiac death. Epicardial coronary arteries involvement is rare, however, coronary angiography should be considered in patients presenting with angina symptoms to rule out important coronary vessels stenosis. Imaging techniques as transthoracic echocardiography (TTE) and cardiac magnetic resonance (CMR) may help in establishing the diagnosis of EGPA. The significance of often detected cardiac damage in CMR without clinical manifestation remains uncertain, however, some preliminary studies suggest that, these patients should be treated more intensively to prevent further cardiac involvement.

The main treatment of EGPA consist of corticosteroids and additional immunosuppression in patients with worse prognosis. In differential diagnosis other forms of vasculitides and hypereosinophilic syndromes (HES) that may also affect the heart should be excluded.


Keywords


cardiac involvement, EGPA, Churg Strauss, cardiac magnetic resonance

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DOI: http://dx.doi.org/10.20418%2Fjrcd.vol1no3.56

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