Milena Bond1, Filippo Fagni2, Michele Moretti3, Federica Bello4, Allyson Egan5, Augusto Vaglio6,7, Giacomo Emmi4, Christian Dejaco8,9. 1. Department of Rheumatology, Hospital of Brunico (SABES-ASDAA), Brunico, Italy. 2. Department of Internal Medicine 3 - Rheumatology and Immunology, Friedrich-Alexander University Erlangen-Nuremberg and Universitätsklinikum Erlangen, Erlangen, Germany. 3. Department of Cardiology - Azienda Provinciale Per I Servizi Sanitari Di Trento, Trento, Italy. 4. Department of Experimental and Clinical Medicine, University of Firenze, and Internal Interdisciplinary Medicine Unit, Careggi University Hospital, Florence, Italy. 5. Vasculitis & Lupus Unit, Department of Medicine, Addenbrookes Hospital, Cambridge, UK. 6. Nephrology and Dialysis Unit, Meyer Children's Hospital, Florence, Italy. 7. Department of Biomedical, Experimental and Clinical Sciences "Mario Serio", University of Firenze, Florence, Italy. 8. Department of Rheumatology, Hospital of Brunico (SABES-ASDAA), Brunico, Italy. christian.dejaco@gmx.net. 9. Department of Rheumatology and Immunology, Medical University of Graz, Graz, Austria. christian.dejaco@gmx.net.
Abstract
PURPOSE OF REVIEW: To provide an overview of existing literature on pathogenetic and clinical aspects of cardiac and vascular involvement in eosinophilic granulomatosis with polyangiitis (EGPA). RECENT FINDINGS: In EGPA, cardiac and vascular involvement are more common than previously thought. However, no international recommendations on the topic are available yet. Herein, we summarize the existing evidence on the topic and propose a diagnostic approach for cardiac involvement in EGPA. The prevalence of cardiovascular involvement in patients with EGPA varies greatly among published studies, ranging between 3.1-18.7% for occlusive arterial disease, 5.8-30% for venous thrombosis and 17-92% for heart involvement. Cardiac involvement in EGPA is associated with high mortality even though manifestations are heterogeneous. In principle, every anatomical structure of the heart can be involved, and EGPA-related heart disease may be completely asymptomatic at first. A careful diagnostic work-up for early detection and prompt treatment initiation is therefore required. While cardiac manifestations are more common in anti-neutrophil cytoplasmic antibodies (ANCA)-negative patients, arterial and venous thrombotic events are not linked to ANCA status but correlate closely with disease activity and accumulate at disease onset. Thrombotic events (mainly venous) are considerably more frequent in EGPA than in the general population contributing substantially to morbidity and highlighting the importance of developing specific prevention strategies for patients who are diagnosed with EGPA.
PURPOSE OF REVIEW: To provide an overview of existing literature on pathogenetic and clinical aspects of cardiac and vascular involvement in eosinophilic granulomatosis with polyangiitis (EGPA). RECENT FINDINGS: In EGPA, cardiac and vascular involvement are more common than previously thought. However, no international recommendations on the topic are available yet. Herein, we summarize the existing evidence on the topic and propose a diagnostic approach for cardiac involvement in EGPA. The prevalence of cardiovascular involvement in patients with EGPA varies greatly among published studies, ranging between 3.1-18.7% for occlusive arterial disease, 5.8-30% for venous thrombosis and 17-92% for heart involvement. Cardiac involvement in EGPA is associated with high mortality even though manifestations are heterogeneous. In principle, every anatomical structure of the heart can be involved, and EGPA-related heart disease may be completely asymptomatic at first. A careful diagnostic work-up for early detection and prompt treatment initiation is therefore required. While cardiac manifestations are more common in anti-neutrophil cytoplasmic antibodies (ANCA)-negative patients, arterial and venous thrombotic events are not linked to ANCA status but correlate closely with disease activity and accumulate at disease onset. Thrombotic events (mainly venous) are considerably more frequent in EGPA than in the general population contributing substantially to morbidity and highlighting the importance of developing specific prevention strategies for patients who are diagnosed with EGPA.
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