A case report and literature review of Churg–Strauss syndrome presenting with myocarditis

Abstract

Background:Churg–Strauss syndrome (CSS) is a multisystem disorder characterized by asthma, prominent peripheral blood eosinophilia, and vasculitis signs.Case summary:Here we report a case of CSS presenting with acute myocarditis and heart failure and review the literature on CSS with cardiac involvement. A 59-year-old man with general fatigue, numbness of limbs, and a 2-year history of asthma was admitted to the department of orthopedics. Eosinophilia, history of asthma, lung infiltrates, peripheral neurological damage, and myocarditis suggested the diagnosis of CSS. Transthoracic echocardiography revealed a dilated hypokinetic left ventricle (left ventricular ejection fraction ∼40%) with mild segmental abnormalities in the septal and apical segments.Conclusion:By reviewing the present case reports, we concluded that (1) the younger age of CSS, the greater occurrence rate of complicating myocarditis and the poorer prognosis; (2) female CSS patients are older than male patients; (3) patients with cardiac involvement usually have a history of severe asthma; (4) markedly increased eosinophil count suggests a potential diagnosis of CSS (when the count increases to 20% of white blood cell counts or 8.1 × 109/L, eosinophils start to infiltrate into myocardium); and (5) negative ANCA status is associated with heart disease in CSS.

Eosinophilic myocarditis as a first presentation of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

“…Whereas ANCA negativity is associated with higher rates of pulmonary and cardiovascular involvement. 1 2 …”

“…His laboratory investigations for haematological mutations associated with primary hypereosinophilia, FIP1L1-PDGFRA and rare BCR-ABL1 variants, and lymphocyte flow cytometry were negative making a diagnosis of HES or chronic eosinophilic leukaemia very unlikely. 1 …”

“…Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is a rare vasculitis affecting small-sized/medium-sized vessels and multiple organs. Symptomatic cardiac involvement occurs in 27% to 47% of cases 1 and can range from palpitations and chest pain to fulminant heart failure and death. The mortality associated with EGPA myocarditis is estimated to be 50%.…”

Myopericarditis as a presentation of eosinophilic granulomatosus with polyangiitis (EGPA)

“…Cardiovascular disease complicated by EGPA is more challenging to treat than a stand-alone disease. Presentations of cardiovascular disease in EGPA may include eosinophilic vasculitis, pericarditis, pericardial effusion, cardiac valve disease, cardiomyopathy and myocarditis, as well as acute myocardial infarction and cardiac failure 8…”

Myocarditis in auto-immune or auto-inflammatory diseases

“…The risk of myocarditis is increased in patients of 20-30 years old (incidence of myocarditis in EGPA patients is 23%). Cardiac manifestations occur later in females than males(21,22). Myocarditis may be associated with a surge in eosinophils, which may suggest the pathogenesis of myocarditis in EGPA patients.…”