Eosinophilic myocarditis in hypereosinophilic syndrome

Roehrl, Michael H. A.; Alexander, Mariam P.; Hammond, Stephen; Ruzinova, Marianna B.; Wang, Julia Y.; O’Hara, Carl

doi:10.1002/ajh.21943

Cited by 15 publications

(10 citation statements)

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“…Although traditionally associated with allergic reactions, eosinophils have a role in tissue remodeling and are found in biopsy specimens from several dermatologic and extracutaneous fibrosing or sclerosing disorders, including ocular pemphigoid, morphea profunda, eosinophilic fasciitis, granuloma faciale, immunoglobulin G4‐related sclerosing diseases, endomyocardial fibrosis associated with hypereosinophilic syndrome, eosinophilic angiocentric fibrosis of the upper respiratory tract and orbit, idiopathic retroperitoneal fibrosis, sclerosing mediastinitis, sclerosing cholangitis and pulmonary fibrosis . Thus, the common presence of tissue eosinophils in LS may be less a diagnostic marker for coexistent allergic or autoimmune disease than a reflection of the sclerosis that is integral to LS.…”

Section: Discussionmentioning

confidence: 99%

Eosinophils in lichen sclerosus et atrophicus

2015

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Section: Discussionmentioning

confidence: 99%

Eosinophils in lichen sclerosus et atrophicus

2015

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“…The initial treatment goal after the diagnosis of EM is to provide hemodynamic stability with standard heart failure medication with full cardio-pulmonary support depending on the severity of heart failure and early treatment with corticosteroids. Systemic corticosteroid is the mainstay of treatment for a patient with EM and it should be commenced as soon as possible [ 16 – 18 ]. The goal of steroid treatment in EM is to reduce eosinophil induced organ damage.…”

Section: Discussionmentioning

confidence: 99%

“…In accordance with this, 85% of patients after 1 month of monotherapy had complete or partial responses. And most patients remained on maintenance doses with a median of 10 mg prednisolone daily dose for 2 months to 20 years [ 17 , 18 ]. Kawano and colleagues were the first to suggest initiation and maintenance doses of prednisolone based on disease severity in EM according to a recent retrospective case series done by them [ 2 ].…”

Section: Discussionmentioning

confidence: 99%

A young female presenting with heart failure secondary to eosinophilic myocarditis: a case report and review of the literature

et al. 2018

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BackgroundEosinophilic myocarditis is one of the fatal complications of idiopathic hypereosinophilic syndromes. Given the rarity of this form of myocarditis, it is often under-recognized. We describe a young girl who presented with features of heart failure. To our knowledge, this is the first reported case of eosinophilic myocarditis in a young Sri Lankan female.Case presentationA previously healthy 21 year old Sri Lankan female admitted with shortness of breath for 1 week duration with associated low grade fever and profuse sweating. She was mildly febrile and dyspnoeic with absent ankle oedema. She was tachycardic and had elevated Jugular venous pressure with negative Kussmaul sign. Blood pressure was 100/70 mmHg. Clinically there was no cardiomegaly and heart sounds were slightly muffled with gallop rhythm. Bilateral basal fine end inspiratory crackles and mild hepatosplenomegaly were noted. The laboratory examinations showed leucocytosis with severe eosinophilia with no abnormal cells. Her ESR, Troponin I and Brain natriuretic peptide were elevated with normal CRP and electrocardiogram showed sinus tachycardia with wide spread ST depression. Heart failure was evident on chest X-ray and 2D-echocardiogram showed global left ventricular hypokinesia with 40% ejection fraction and a thin layer of pericardial effusion. Mild hepatosplenomegaly without lymphadenopathy was detected in the ultrasound scan. Bone marrow biopsy showed hypereosinophilia with no evidence of bone marrow infiltration. FIP1L1–PDGFRA fusion transcript and BCR–ABL transcript were not detected. Secondary causes for hypereosinophilia were excluded and the diagnosis of idiopathic hypereosinophilic syndrome and eosinophilic myocarditis was made. She had good response to steroids clinically and biochemically with complete recovery of left ventricular function. She is now on steroid to be continued at least 6 months to 1 year.ConclusionEosinophilic myocarditis is a rare but fatal disease if left untreated. Hence clinicians should have high index of suspicion to diagnose eosinophilic myocarditis in clinical context of heart failure due to myocarditis. The diagnoses of eosinophilic myocarditis may often be challenged especially in a poor recourse setting. However available investigation should be used to diagnose this condition without delay. Early treatment with systemic steroids may prevent fatal outcome and therapies for this disease have yet to be validated in large prospective studies.

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“…However some case series report cardiac involvement in up to 40-50% of patients with HES [31]. Corticosteroid therapy in HES has been successfully documented in published case reports with induced complete or partial responses at 1 month in 85% of patients following monotherapy; most patients remained on maintenance doses with a median of 10 mg prednisone equivalent daily dose for 2 months to 20 years [4,30,32]. It remains uncertain however why our patients failed to respond to the proposed therapy.…”

Section: Discussionmentioning

confidence: 99%

Eosinophilic myocarditis: two case reports and review of the literature

et al. 2013

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BackgroundEosinophilic myocarditis is a rare and often under-diagnosed subtype of myocarditis with only around 30 cases published in the medical literature. In this article we present two patients with eosinophilic myocarditis with the aim to demonstrate the often elusive nature of the disease and present the current scientific literature on this topic.Case presentationA 76 years old Caucasian gentleman and a 36 years old Aboriginal gentleman both presenting with heart failure symptoms were eventually diagnosed with eosinophilic myocarditis after extensive evaluation. Their presentation, assessment, and medical management is explored in this article.ConclusionsEosinophilic myocarditis remains a rare and likely under-diagnosed subtype of myocarditis. The key features of this disease include myocardial injury in the setting of non-contributory coronary artery disease. Endomyocardial biopsy remains the definitive gold standard for diagnosis of noninfectious eosinophilic myocarditis. Non-invasive cardiac imaging in the setting of peripheral eosinophilia can be strongly suggestive of eosinophilic myocarditis with potential for earlier diagnosis. Failure to diagnose eosinophilic myocarditis and the delay of therapy may lead to irreversible myocardial injury. Therapies for this disease have yet to be validated in large prospective studies.

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Eosinophilic myocarditis in hypereosinophilic syndrome

Cited by 15 publications

References 3 publications

Eosinophils in lichen sclerosus et atrophicus

Eosinophils in lichen sclerosus et atrophicus

A young female presenting with heart failure secondary to eosinophilic myocarditis: a case report and review of the literature

Eosinophilic myocarditis: two case reports and review of the literature

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