Cardiac Involvement of Churg-Strauss Syndrome as a Reversible Cause of Dilated Cardiomyopathy

Abstract

A 31-year-old male who had been treated for Churg-Strauss syndrome (CSS) presented with sudden onset of dysarthria. Brain magnetic resonance imaging (MRI) showed acute multifocal bilateral cerebral infarctions suggesting embolic causes. Cardiac MRI showed dilated cardiomyopathy with severe biventricular dysfunction with intracardiac thrombi, and multiple high signal intensity spots in myocardium of the left ventricle with multifocal delayed enhancement suggesting multifocal myocarditis due to small vessel vasculitis associated with CSS. After anticoagulation therapy, treatments for heart failure, and immunosuppressive therapy including parenteral steroids and cyclophosphamide to control CSS, the symptoms and signs of heart failure and cardiac function of the patient were improved. Considering the pathophysiologic mechanism of cardiac involvement in CSS, immunosuppressive therapy to control the disease activity of CSS should be taken into account, besides usual management for heart failure.

Systemic autoimmunity induced by the TLR7/8 agonist Resiquimod causes myocarditis and dilated cardiomyopathy in a new mouse model of autoimmune heart disease

“…These features of myocardial dysfunction and LV dilation with largely unchanged LV mass are indicative of dilated cardiomyopathy, and have been observed in patients with underlying autoimmune disease. Dilated cardiomyopathy can develop secondary to myocarditis ( Cihakova and Rose, 2008 ; Eriksson and Penninger, 2005 ), and has been observed in SLE, systemic sclerosis, dermatomyositis and Churg–Strauss syndrome ( Chen et al, 2014 ; Elliott, 2000 ; Jeong et al, 2015 ; Quartier et al, 2002 ; Zhang et al, 2012 ).…”

“…Resiquimod-induced systemic autoimmunity caused a morphological and functional cardiac phenotype that resembled immune-mediated myocarditis and dilated cardiomyopathy in a variety of autoimmune conditions, including SLE, systemic sclerosis, dermatomyositis and Churg–Strauss syndrome ( Chen et al, 2014 ; Elliott, 2000 ; Jeong et al, 2015 ; Quartier et al, 2002 ; Zhang et al, 2012 ).…”

Angina in granulomatosis with polyangiitis: A case report

“…Approximately 6–44% of granulomatous vasculitis patients have comorbid pericarditis, myocarditis and coronary arteritis with cardiac involvement; of these, 50% have coronary artery vasculitis. In patients with atherosclerosis, GPA can accelerate the process, with a significant increase in morbidity and mortality due to cardiovascular disease, and a poor prognosis ( 6 – 8 ).…”

Cardiac manifestations in antineutrophil cytoplasmic autoantibody (ANCA) — associated vasculitides

“…There are some cases in literature describing dilated cardiomyopathy in patients with EGPA [54,55]. Jeong et al [56] presented a case of reversible dilated cardiomyopathy and intracardiac thrombi, which was diagnosed by MRI in an EGPA patient presenting with multifocal cerebral infarction [56]. Ischaemia and MI in EGPA patients are combined with the inflammatory process and eosinophil accumulation, with epicardial coronary arteries rarely demonstrating any changes during the coronary angiography [57].…”

“…Therapy with high-dose corticosteroids plus immunosuppressive agents, particularly cyclophosphamide in case of myocardial inflammation, is a life-saving procedure [58]. The use of immunosuppressives to control the activity of EGPA in addition to conventional heart failure medical therapy should be considered in patients with depressed left ventricle systolic function due to myocarditis [56].…”