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Cardiac Involvement in Eosinophilic Granulomatosis with Polyangiitis
Mukunthan Srikantharajah1,2, Deepa Gopalan3,4, Harold Wilson-Morkeh5,6
1Vasculitis Centre, Department of Immunology and Inflammation, Imperial College London, London, UK.
Insights
Cardiac disease significantly impacts patients with Eosinophilic granulomatosis with polyangiitis (EGPA), causing approximately 50% of deaths. Early diagnosis using cardiac magnetic resonance (CMR) is crucial for timely treatment and improved outcomes.
Area of Science:
- Cardiology
- Rheumatology
- Immunology
Background:
- Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis.
- Cardiac involvement is a leading cause of mortality in EGPA patients.
- Current diagnostic criteria for cardiac disease in EGPA are not standardized.
Purpose of the Study:
- To review recent advancements in understanding cardiac disease in EGPA.
- To outline a diagnostic approach for early identification of cardiac involvement.
- To emphasize the importance of cardiac assessment in EGPA management.
Main Methods:
- Review of recent literature on EGPA and cardiac disease.
- Highlighting the role of cardiac magnetic resonance (CMR) in diagnosis.
- Discussion of diagnostic challenges and proposed strategies.
Main Results:
- CMR can detect cardiac abnormalities in EGPA patients, even those asymptomatic.
- Cardiac disease is prevalent and a major contributor to mortality in EGPA.
- Systemic immunosuppressive therapy is essential for managing cardiac complications.
Conclusions:
- Early detection of cardiac disease in EGPA is critical.
- Cardiac magnetic resonance (CMR) should be part of the baseline evaluation for all EGPA patients.
- Prompt immunosuppressive therapy can mitigate acute and long-term cardiac damage.
Purpose Of Review:
This review highlights recent advances in the pathophysiology, diagnosis, and treatment of cardiac disease in patients with Eosinophilic granulomatosis with polyangiitis (EGPA). We outline a diagnostic approach to facilitate early identification of affected patients.
Recent Findings:
Recent advancements in diagnostic techniques including cardiac magnetic resonance (CMR) have improved recognition of cardiac disease in patients with EGPA. CMR has demonstrated a high prevalence of cardiac abnormalities, even in the absence of clinical symptoms, electrocardiographic or echocardiographic findings. Cardiac disease is a major cause of mortality in patients with EGPA, accounting for around 50% of disease-related deaths. However, due to the lack of standardised screening and diagnostic criteria, the true incidence remains unknown. Systemic immunosuppressive therapy is warranted to prevent acute complications as well as mitigate the long-term impact of chronic inflammation and tissue damage. Given the challenges in early detection and the prognostic significance of cardiac involvement, we recommend including CMR in the baseline evaluation of all EGPA patients at diagnosis.
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