[Usefulness of magnetic resonance imaging in Churg-Strauss syndrome related cardiac involvement. A case series of three patients and literature review]

Abstract

Purpose: The reported prevalence of cardiac complications is variable in patients with Churg-Strauss syndrome (15-92%) and depends on diagnostic tools. Diagnosis at early stage of heart involvement is crucial, resulting in appropriate management.

Methods: We report three patients who developed cardiac manifestations, revealing Churg-Strauss syndrome. The diagnosis of cardiac involvement was obtained using cardiac magnetic resonance imaging (MRI).

Results: Two patients were males and the remaining one was a female. Presenting clinical manifestations were: cardiac failure (n=1) and retrosternal pain (n=2). Laboratory findings disclosed: high blood count of eosinophils (range: 6000-11,000/mm(3)); antineutrophil cytoplasmic antibodies were positive in a single patient. Cardiac MRI demonstrated: (1) late gadolinium enhancement (n=3), involving mainly the apical and mid-cavity left ventricular segments; (2) impaired left ventricular function (n=2), mean left ventricular ejection fraction being: 51%; and (3) pericardial effusion (n=3). Outcome was favourable after institution of combined therapy with prednisone and cyclophosphamide (n=2); one patient also underwent plasma exchanges.

Conclusion: Our case series underlines that MRI is a helpful tool in the diagnosis of Churg-Strauss syndrome-related cardiac complications. We further suggest that clinical assessment of patients with Churg-Strauss syndrome should include cardiac MRI, in order to detect cardiac involvement at an early stage; indeed, because cardiac manifestations are predictive factors of poor prognosis, diagnosis at early stages of cardiac involvement may result in improvement of patients management.