Churg-Strauss Syndrome Complicated by Eosinophilic Endomyocarditis

Ramakrishna, Gautam; Connolly, Heidi M.; Tazelaar, Henry D.; Mullany, Charles J.; Midthun, David E.

doi:10.4065/75.6.631

Cited by 40 publications

(28 citation statements)

References 22 publications

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“…The myocardial damage is caused by vasculitis leading to coronary arteritis and coronary occlusion, through the release by activated eosinophils of toxic mediators causing direct myocardial damage, 12 or by replacement of the myocardium with granulomas and scar tissue. 13,14 Patients with cardiac involvement are mainly ANCA-negative, as in this case. 15 In general, the prognosis of CSS is good, with an overall 10-year survival of 81-92%.…”

Section: Discussionmentioning

confidence: 71%

Churg–Strauss syndrome presenting with eosinophilic myocarditis: A diagnostic challenge

Correia¹,

Gonçalves²,

Araújo³

et al. 2013

Revista Portuguesa de Cardiologia

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Churg-Strauss syndrome (CSS) is an unusual disease that presents as systemic vasculitis and peripheral eosinophilia in patients with an atopic constitution. Cardiac involvement is unusual and often not prominent on initial presentation, but is an important cause of morbidity and mortality in patients with CSS. We report the case of a young woman with severe acute myocarditis. Coronary arteriography demonstrated extensive focal vasculopathy, consistent with coronary vasculitis, and myocardial biopsy showed eosinophilic myocarditis. This presentation led to an initial diagnosis of CSS in this patient and appropriate therapy resulted in a spectacular remission of disease activity.

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Section: Discussionmentioning

confidence: 71%

Churg–Strauss syndrome presenting with eosinophilic myocarditis: A diagnostic challenge

Correia¹,

Gonçalves²,

Araújo³

et al. 2013

Revista Portuguesa de Cardiologia

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“…However, as with the present case, documented cases of eosinophilic myocarditis due to Churg-Strauss syndrome are relatively rare. 4,5) Our patient had remarkable hypereosinophilia (> 10,000/μL), which is an uncommon feature in Churg-Strauss syndrome with or without cardiac involvement. 4,6) This uncommon feature may explain the markedly elevated ECP in this case.…”

Section: Discussionmentioning

confidence: 82%

Eosinophilic Myocarditis due to Churg-Strauss Syndrome With Markedly Elevated Eosinophil Cationic Protein

et al. 2013

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SummaryA 67-year-old woman with asthma visited our hospital with increasing dyspnea and new-onset paresthesia and purpura in her legs. Physical examination showed a wheeze, pretibial edema, and surrounding purpura. Chest X-rays showed cardiac decompensation and an electrocardiogram revealed a new ST-T change. Laboratory data showed leukocytosis, hypereosinophilia (10,450/μL), troponin T(+), elevated BNP, and markedly elevated eosinophil cationic protein (ECP) (> 150 ng/mL). Echocardiography revealed diffuse left ventricular hypokinesis (ejection fraction 30%) with increased wall thickness. Coronary angiography was normal. Cardiac magnetic resonance imaging implied diffuse myocardial edema and subendocardial late gadolinium enhancement. Skin biopsy of purpura showed superfi cial perivascular dermatitis with remarkable eosinophilic infi ltrations. No evidence of drug allergies, parasitic infection, or myeloproliferative disorder was detected. Based on these fi ndings, a diagnosis of eosinophilic myocarditis due to Churg-Strauss syndrome was considered. She was administered prednisolone at a dose of 1 mg/kg, cyclophosphamide, and diuretics. Several markers of eosinophilic myocarditis and heart failure gradually improved, including ECP. She was discharged 30 days later with no cardiac event. Eosinophilic myocarditis is characterized by predominantly eosinophilic infi ltration. Eosinophilic granule proteins, such as ECP and major basic protein, play important roles in the pathogenesis of eosinophilic myocarditis. We experienced a rare case of eosinophilic myocarditis due to Churg-Strauss syndrome. Markedly elevated ECP played an important role in the early diagnosis and subsequent reduction in ECP served as a marker of monitoring. In an asthmatic patient with dyspnea, hypereosinophilia, and vasculitis, Churg-Strauss syndrome with eosinophilic myocarditis should be considered. (Int Heart J 2013; 54: 51-53) Key words: Heart failure, Infl ammation E osinophilic myocarditis is characterized by predominantly eosinophilic infiltration. Eosinophilic granule proteins, such as eosinophil cationic protein (ECP) and major basic protein (MBP), play important roles in the pathogenesis of eosinophilic myocarditis. It may occur in a variety of settings; allergic disease (eg, allergic rhinoconjunctivitis and asthma), drug reactions, parasitic infection, malignancies (eg, lymphoma), and systemic disorders (eg, idiopathic hypereosinophilic syndromes and vasculitis). Here we report a rare case of eosinophilic myocarditis due to Churg-Strauss syndrome with markedly elevated ECP. Case ReportA 67-year-old woman with asthma presented to our hospital with increasing dyspnea and new-onset paresthesia and purpura in her legs ( Figure 1A). She had no history of hypertension or heart disease. Her arterial blood pressure was 120/84 mmHg, pulse rate 108 bpm, body temperature 37.1°C, and arterial saturation 97% (pernasal, 2L). A wheeze was present in the bilateral lung fi elds and gallop rhythm was audible. Bilateral pretibial edema and surroundin...

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“…Ischemia or infarction in ChurgStrauss syndrome is typically related to inflammation of small distal coronary vessels, although vasculitis of epicardial coronary arteries has been reported with patients presenting with acute coronary syndromes or myocardial infarctions (MIs) [5]. In addition to predisposing to arterial thromboembolus, endocardial thickening and thrombus may extend down the myocardial surface to engulf the valvular apparatus or leaflets, classically affecting the atrioventricular valves, leading to significant valve dysfunction that may not be amenable to medical therapy alone [6].…”

Section: Myocardial and Valvular Vasculitic Involvement In Antineutromentioning

confidence: 99%

Involvement of the heart by small and medium vessel vasculitis

Kane¹,

Keogh²

2009

Current Opinion in Rheumatology

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Most small and medium vessel vasculitides may involve the heart; however, the mode and incidence of cardiac involvement vary with the different vasculitic syndromes. This review describes the various cardiac manifestations of small and medium vessel vasculitis and the advantages of modern imaging modalities including echocardiography, MRI, and PET coupled with biologic biomarkers such as brain natriuretic peptide and antineutrophilic cytoplasmic antibodies in the diagnosis and management of disease.

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Churg-Strauss Syndrome Complicated by Eosinophilic Endomyocarditis

Cited by 40 publications

References 22 publications

Churg–Strauss syndrome presenting with eosinophilic myocarditis: A diagnostic challenge

Churg–Strauss syndrome presenting with eosinophilic myocarditis: A diagnostic challenge

Eosinophilic Myocarditis due to Churg-Strauss Syndrome With Markedly Elevated Eosinophil Cationic Protein

Involvement of the heart by small and medium vessel vasculitis

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