Multimodality Assessment of Cardiac Involvement in Churg-Strauss Syndrome Patients in Clinical Remission

Szczeklik, Wojciech; Miszalski−Jamka, Tomasz; Mastalerz, Lucyna; Sokołowska, Barbara; Dropiński, Jerzy; Banyś, Robert; Hor, Kan N.; Mazur, Wojciech; Musiał, Jacek

doi:10.1253/circj.cj-10-0772

Cited by 61 publications

(47 citation statements)

References 34 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Inflammation/edema and fibrosis -perhaps reflecting ''healed'' inflammation and accounting for diastolic dysfunction and restrictive cardiomyopathy -can coexist but are not always possible to be appropriately classified with traditional echocardiography [2]. Deformation parameters, although not established for diagnostic purposes, may provide useful insight in the characterization of tissue mechanics reflecting histopathological changes of CSS even in subclinical disease.…”

Section: Discussionmentioning

confidence: 99%

A Churg–Strauss syndrome patient with myopericardial involvement

Papadimitraki

Moyssakis

Mavrogeni

et al. 2015

Journal of Cardiology Cases

View full text Add to dashboard Cite

IntroductionChurg-Strauss syndrome (CSS) is a rare necrotizing vasculitis of small vessels. Late onset asthma, nasal polyps, and sinusitis are early manifestations with eosinophilia and vasculitic tissue affecting nervous system, gastrointestinal tract, skin, and lungs, with heart involvement occurring later in the disease course. Case reportHerein we present the case of a 45-year-old woman with CSS and history of asthma and nasal polyps, who presented with dyspnea (New York Heart Association III) of recent onset. On admission, the patient was in respiratory distress with blood pressure 110/70 mmHg and inspiratory crackles. Her chest-X-ray revealed bilateral pleural effusions and the electrocardiogram showed sinus tachycardia of 100 bpm, left ventricular hypertrophy, and diffuse horizontal ST segment depression. Blood analysis was remarkable for severe eosinophilia (15,000/ml), white blood cells 24,000/ml, abnormal troponin (1.5 ng/ml), and B-type natriuretic peptide 800 pg/ml. Initial transthoracic echocardiogram (TTE) revealed a left ventricle (LV) of normal internal dimensions with mild systolic dysfunction (ejection fraction = 45%), hypokinetic inferior and inferolateral and mid/apical anteroseptal segments. There was marked hyperechogenicity of inferior and inferolateral wall involving both the subendocardial and subepicardial segments. An accompanying moderate pericardial effusion causing protodiastolic compression of the right ventricle was present. Mitral and tricuspid annular infiltrations with moderate valvular regurgitation were evident as well. Right ventricular hypertrophy with reduced tricuspid annular systolic velocity (7 cm/sec) was also found. There were no intraventricular thrombi. Interestingly, both global longitudinal and circumferential LV strain were severely impaired (À12% and À8%, respectively) (Fig. 1a, c and e).The patient received cyclophosphamide (CYC) monthly doses of 2 g for six consecutive months and steroids (1 g methylprednisolone/24 h Â 3 days switched to prednisolone with progressive tapering) with clinical improvement soon after the first infusion.

show abstract

Section: Discussionmentioning

confidence: 99%

A Churg–Strauss syndrome patient with myopericardial involvement

Papadimitraki

Moyssakis

Mavrogeni

et al. 2015

Journal of Cardiology Cases

View full text Add to dashboard Cite

show abstract

“…26,27 Since cardiac involvement is one of the most important predictors of an adverse outcome, 18 these findings could influence current recommendations for cardiac evaluation and therapeutic decisions in asymptomatic patients. An early diagnosis detecting silent inflammation could be valuable, 28 since appropriate therapy can prevent progression to harmful episodes of cardiac disease and so reduce the high mortality associated with these manifestations.…”

Section: Discussionmentioning

confidence: 99%

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

Correia¹,

Gonçalves²,

Araújo³

et al. 2013

Revista Portuguesa de Cardiologia

View full text Add to dashboard Cite

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.

show abstract

“…Recent studies have demonstrated that cardiac MRI might be the most sensitive method for early detection and serial follow-up of cardiac involvement in EGPA patients [60,61]. MRI can demonstrate not only the involvement of whole myocardium of all cardiac chambers, but also pericardial involvement.…”

Section: Cardiovascular Multimodality Imagingmentioning

confidence: 99%