Dataset Information

Eosinophilic perimyocarditis associated with eosinophilic granulomatosis with polyangiitis: a case report.

ABSTRACT:

Background

Eosinophilic myocarditis (EM) is a rare and potentially life-threatening form of myocarditis, frequently (but not always) associated with eosinophilia, and presents with acute chest pain, or signs and symptoms of acute or chronic heart failure. Eosinophilic myocarditis has various aetiologies, including eosinophilic granulomatosis with polyangiitis (EGPA).

Case summary

A 52-year-old female with a long-standing history of asthma, acral paraesthesia, subcutaneous nodules, and recurrent chest pain treated with anti-inflammatory drugs was admitted to our hospital with chest pain, repolarization disturbances, eosinophilia, and increased troponin levels. After an initial evaluation by coronary angiography, echocardiography and cardiac magnetic resonance, a definitive diagnosis of EM was made with the help of an endomyocardial biopsy. The aetiological diagnosis of EM as a manifestation of tissue involvement in EGPA was concluded after ruling out other possible causes of eosinophilia and with the help of other diagnostic criteria for EGPA (asthma, eosinophilia, and neuropathy). Therefore, we started with a high dosage of glucocorticoids, and attained relief of symptoms and normalization of eosinophilic count after a few days.

Discussion

In cases of myocarditis (particularly if associated with eosinophilia), EM is a manifestation of EGPA and should be considered for a prompt differential diagnosis. Endomyocardial biopsy represents the gold standard for the diagnosis of EM. The mainstay of therapy for EM is immunosuppressive drugs to help prevent its evolution to a fulminant form and chronic progression towards restrictive cardiomyopathy.

SUBMITTER: Blumetti L

PROVIDER: S-EPMC8564711 | biostudies-literature | 2021 Oct

REPOSITORIES: biostudies-literature

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Publications

Eosinophilic perimyocarditis associated with eosinophilic granulomatosis with polyangiitis: a case report.

Blumetti Ludovica L De Perna Maria Luisa ML Reinehr Michael M Pedrazzini Giovanni G

European heart journal. Case reports 20211007 10

<h4>Background</h4>Eosinophilic myocarditis (EM) is a rare and potentially life-threatening form of myocarditis, frequently (but not always) associated with eosinophilia, and presents with acute chest pain, or signs and symptoms of acute or chronic heart failure. Eosinophilic myocarditis has various aetiologies, including eosinophilic granulomatosis with polyangiitis (EGPA).<h4>Case summary</h4>A 52-year-old female with a long-standing history of asthma, acral paraesthesia, subcutaneous nodules, ...[more]

PMID: 34738066

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Project description:BackgroundPatients with asthma exhibit a significantly heightened susceptibility to eosinophilic granulomatosis with polyangiitis (EGPA) when compared to the general population. Vigilance for EGPA manifestations is crucial, especially in cases where asthma remains poorly controlled despite high-dose corticosteroid therapy or when eosinophil counts exceed 5%. The diagnosis of EGPA can be complex due to the absence of definitive biomarkers, as indicated by the American College of Rheumatology (ACR)'s 1990 classification criteria. EGPA is categorized as an antineutrophil cytoplasmic antibody (ANCA) associated vasculitis, with updated classification criteria released in 2022, which require a cumulative score of 6 or more for the diagnosis of small and medium vessel vasculitis. Enhancing knowledge of EGPA facilitates its early detection and effective management.Case descriptionThe patient was initially diagnosed with allergic rhinitis in 2006 and developed cough and wheezing in 2016. In 2017, EGPA was diagnosed based on ACR criteria, with a cumulative score of 14 according to the 2022 ACR and the European League Against Rheumatism (ACR/EULAR) criteria, indicating small and medium vessel vasculitis. The patient showed myocardial, gastric, and neurological involvement, reflecting generalized EGPA. Prognostic assessments should use the five-factor score (FFS), which indicates a 46% 5-year mortality rate for those with an FFS of 2 or higher. This patient had an FFS of 3, tested negative for ANCA, and cardiac emission computed tomography (ECT) confirmed myocardial involvement. However, as EGPA was diagnosed only 13 months after the onset of wheezing, the patient had been undergoing glucocorticoid therapy, as of today (7 years later), has effectively managed the symptoms and facilitated normal daily activities.ConclusionsIf asthma symptoms persist despite intensive corticosteroid treatment or the eosinophil count exceeds 5%, consider the possibility of EGPA. The presence of ANCA exerts a substantial impact on the prognostic outcomes in EGPA. ANCA-negative patients typically exhibit reduced survival rates, primarily attributed to a higher incidence of cardiac involvement. Nevertheless, advancements in early diagnosis and therapeutic interventions have led to improved survival rates, even in cases complicated by cardiac and pulmonary manifestations.

Dataset Information

Eosinophilic perimyocarditis associated with eosinophilic granulomatosis with polyangiitis: a case report.

Background

Case summary

Discussion

Publications

Eosinophilic perimyocarditis associated with eosinophilic granulomatosis with polyangiitis: a case report.

Similar Datasets

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