Successfully treated eosinophilic granulomatosis with polyangiitis relapse presenting as myocarditis and followed by multimodality imaging

Miyazaki, Motonobu; Hattori, Hidetoshi; Suzuki, Atsushi; Serizawa, Naoki; Uto, Kenta; Fukui, Kenji; Momose, Mitsuru; Shiga, Tsuyoshi; Hagiwara, Nobuhisa

doi:10.1016/j.jccase.2018.06.008

Cited by 5 publications

(3 citation statements)

References 6 publications

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“…22 A CMRI study targeted to detect cardiac lesions and to monitor the treatment efficacy in EGPA patients with cardiac involvement revealed the presence of myocardial edema in 87.8% patients, perfusion defects in 54.5%, and late gadolinium enhancement indicative of replacement fibrosis, in all patients. 19 In our case, CMRI lesions were typical of EGPA and similar to other described cases, [28][29][30] but they are extensive and simultaneously affected all layers of the heart. The lesions included the myocardium, endocardium, and pericardium, the formation of wall thrombi at the apex of the left ventricle, and reduced the leftventricular EF.…”

Section: Discussionsupporting

confidence: 83%

Cardiac Involvement and Absence of Asthma – What is Phenotype Specificity of EGPA: A Case Report

Fijołek¹,

Wiatr²,

Błasińska³

et al. 2022

Clin Med Res

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Eosinophilic granulomatosis with polyangiitis (EGPA) typically is characterized by asthma, blood eosinophilia, and extrapulmonary manifestations. Asthma is a major EGPA symptom affecting almost all patients, but cardiac involvement is one of the most serious manifestations, responsible for 31% of deaths. Two recently defined phenotypes of EGPA, according to the patient's antineutrophil cytoplasmic antibody (ANCA) status, differ significantly in clinical features and prognosis. We share a case of EGPA characterized by atypical manifestation without any evidence of asthma, in whom extensive cardiac involvement was the dominant manifestation of vasculitis. This case demonstrates the difficulties associated with phenotyping EGPA and highlights the importance of cardiac magnetic resonance imaging (CMRI) in definitive diagnosis.

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Section: Discussionsupporting

confidence: 83%

Cardiac Involvement and Absence of Asthma – What is Phenotype Specificity of EGPA: A Case Report

Fijołek¹,

Wiatr²,

Błasińska³

et al. 2022

Clin Med Res

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“…Notably, in our case, multiple foci on the initial LGE were resolved following the corticosteroid treatment. Similar foci have been reported in some cases of EGPA-associated EM, and are presumed to be specific to vasculitis, which can be an indicator for EGPA-associated EM ( 35 , 43 , 55 ). Further studies on the relationship between foci on LGE and their respective pathologies are warranted.…”

Section: Discussionsupporting

confidence: 78%

The Diagnostic Challenge of Eosinophilic Granulomatosis With Polyangiitis Presenting as Acute Eosinophilic Myocarditis: Case Report and Literature Review

Yamamoto

HASHIMOTO

Ikeda

et al. 2022

Front. Cardiovasc. Med.

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Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis involving small-to-medium-sized vessels characterized by asthma, vasculitis, and peripheral eosinophilia. EGPA-associated eosinophilic myocarditis (EM) occurs rarely, yet can be fatal if left untreated. Moreover, the accurate diagnosis of EGPA-associated EM without vasculitis is exceptionally difficult because of the overlapping features with EM of other causes. We report a case of probable EGPA with subclinical neurological involvement that presented with acute EM. The constellation of peripheral eosinophilia, left ventricular dysfunction, and normal epicardial coronary arteries raised suspicion of acute EM, which was confirmed by cardiac magnetic resonance (CMR) investigation and endomyocardial biopsy (EMB). Prompt systemic administration of corticosteroids completely restored and normalized myocardial structure and function. Although the patient's history suggested the presumed hypersensitivity myocarditis, EMB revealed EM without vasculitis, not hypersensitivity, leading to a tentative diagnosis of idiopathic hypereosinophilic syndrome. Interestingly, the characteristic findings of vasculitis on CMR imaging strongly suggested EGPA-associated EM. Although the patient had no clinical neurological manifestations, a nerve conduction study confirmed mononeuritis multiplex, leading to the final diagnosis of probable EGPA. Therefore, this case highlights the diagnostic challenge associated with EGPA and the diagnostic synergy of CMR and EMB for an exploratory diagnosis of EGPA-associated EM.

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“…Untreated cardiac deficits are associated with increased mortality and poor prognosis. CMR, fluorodeoxyglucose positron emission tomography (FDG-PET) scan, echocardiography (ECG), and endomyocardial biopsy (EMB) are standard methods of detection of cardiac pathology [ 5 , 7 - 10 ]. ECG is the only technique part of the regular protocol currently used in EGPA patient management.…”

Section: Introductionmentioning

confidence: 99%

The Critical Role of Cardiac Magnetic Resonance Imaging in Evaluating Patients With Eosinophilic Granulomatosis With Polyangiitis

Sridharan

Nanthakumaran

Somagutta

et al. 2020

Cureus

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Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare autoimmune systemic necrotizing vasculitis of blood vessels that often presents with hypereosinophilia. Cardiac involvement in EGPA directly correlates with the mortality of patients with the disease and is a central part of the disease process. The evaluation and treatment of cardiac anomalies are vital in patients with EGPA. The frequency with which cardiac involvement is seen in the disease process makes early diagnosis crucial in all patients with EGPA. Early treatment has been proven to reverse or cause the disease to go into remission. Several studies have shown that cardiac magnetic resonance (CMR) imaging is the most sensitive and best early indicator of cardiovascular involvement in EGPA. CMR routinely outperforms other diagnostic techniques such as ECG (echocardiography) and CTA (computed tomography angiography) in the detection of cardiac anomalies and should be a part of the standardized assessment of all patients with EGPA. CMR is also a non-invasive diagnostic tool that can also outperform biopsy in the detection of EGPA cardiac involvement. CMR is also a valuable technique that can be used to monitor disease progression while treatment is being performed. Although long-term research studies have yet to show these benefits, the studies that are available today provide ample evidence that shows CMR imaging could ultimately help bring down mortality rates currently seen in EGPA patients if it is used as an evaluation tool from initial diagnosis and throughout the entire course of disease management.

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Successfully treated eosinophilic granulomatosis with polyangiitis relapse presenting as myocarditis and followed by multimodality imaging

Cited by 5 publications

References 6 publications

Cardiac Involvement and Absence of Asthma – What is Phenotype Specificity of EGPA: A Case Report

Cardiac Involvement and Absence of Asthma – What is Phenotype Specificity of EGPA: A Case Report

The Diagnostic Challenge of Eosinophilic Granulomatosis With Polyangiitis Presenting as Acute Eosinophilic Myocarditis: Case Report and Literature Review

The Critical Role of Cardiac Magnetic Resonance Imaging in Evaluating Patients With Eosinophilic Granulomatosis With Polyangiitis

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