Israel Medical Association Journal

Eosinophilic myocarditis (EM) is a rare form of myocardial inflammation defined by eosinophilic infiltration with associated myocyte injury. Its clinical expression is heterogeneous, ranging from subtle symptoms of heart failure to fulminant cardiogenic shock. Because these features often overlap with other types of myocarditis and systemic conditions, establishing the diagnosis can be difficult. In such cases, endomyocardial biopsy (EMB) remains the reference standard.

Amyloid transthyretin (ATTR) cardiac amyloidosis is an infiltrative cardiomyopathy marked by amyloid fibril deposition in the heart's extracellular space. It usually presents with signs of heart failure, and echocardiography often shows concentric left ventricular hypertrophy (LVH), restrictive physiology, and a distinctive pattern of apical strain sparing. In recent years, the development of non-invasive diagnostic methods, as well as the availability of disease-modifying treatments, has significantly improved diagnosis and management.

To the best of our knowledge, the coexistence of ATTR cardiac amyloidosis and EM has not been previously reported. Such overlap presents distinct diagnostic and therapeutic challenges. These conditions may have synergistic effects on the structure and function of the myocardium. We describe a case of concurrent ATTR cardiac amyloidosis and EM, highlighting the role of multimodality imaging and multidisciplinary management of these overlapping pathologies.

Idiopathic eosinophilic vasculitis is a newly recognized form of hypereosinophilic syndrome. While little is understood about the condition, criteria for its definition have been proposed. We aimed to determine whether three patients with eosinophilia and vasculitis could be retrospectively diagnosed with this condition. We performed a retrospective descriptive analysis on three cases with hypereosinophilia and vasculitis who were treated in Sheba Medical Center, Sourasky Medical Center, and Meir Medical Center in Israel between 2009 and 2021. A thorough review of all three cases was conducted. The findings were compared to the suggested criteria for idiopathic eosinophilic vasculitis.

All patients shared the symptoms of progressive limb ischemia, eosinophilic rash, and peripheral neuropathy that are consistent with vasculitis. No lower or upper respiratory abnormalities or the presence of anti-neutrophil cytoplasmic antibodies (ANCA) autoantibodies associated with eosinophilic granulomatosis with polyangiitis were detected. Primary monoclonal abnormalities, drug interactions, infections, allergy, and other secondary causes of hypereosinophilia were excluded. After a thorough review, we suggest that our three patients with previously unexplained hypereosinophilia and vasculitis fit the diagnosis of idiopathic eosinophilic vasculitis. These results highlight the existence of this novel condition and the importance of its recognition and consideration as part of the differential diagnosis in patients with marked eosinophilia and vasculitis. Further research for elucidating the mechanisms and treatment approach for this potentially severe condition is urgently needed.

Hypereosinophilia is defined as the absolute eosinophilic count of above 1500 cells/µL in the peripheral blood on two separate tests taken during one month and/or the pathological confirmation of hypereosinophilia. There are many conditions that are associated with increased eosinophil counts including: parasitic infections, drug reactions, eosinophilic granulomatosis with polyangiitis, allergic reactions, drug reaction with eosinophilia and systemic symptoms (DRESS), primary immunodeficiencies (PID), eosinophilic gastrointestinal diseases (EGID), familial hypereosinophilia, and neoplasms [1]. Molecular classification may be an adjuvant in the classification of hypereosinophilia [2]. Our patient presented with hypereosinophilia as part of a paraneoplastic syndrome.