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[Heart failure as clinical onset of essential mixed cryoglobulinemia]
G Bragagni1, A Baldini, M Bianconcini
1Ospedale di Cento (Ferrara), Divisione di Medicina Interna, Azienda USL, Ferrara. giambra@id.it
Insights
Essential mixed cryoglobulinemia (EMC) can present as heart failure due to coronary microcirculation issues, not just myocardial infarction. This case highlights EMC
Area of Science:
- Cardiology
- Rheumatology
- Internal Medicine
Background:
- Essential mixed cryoglobulinemia (EMC) is a rare systemic vasculitis.
- Cardiac involvement in EMC is uncommon, typically presenting as myocardial infarction.
- Heart failure is a known complication and cause of mortality in EMC patients.
Observation:
- A 58-year-old man presented with heart failure as the primary symptom of EMC.
- Extensive diagnostic workup, including cardiac imaging and biopsies, confirmed EMC.
- The patient exhibited myocardial contractile deficiency on an ischemic basis despite a healthy coronary artery system.
Findings:
- Heart failure was attributed to coronary microcirculation impairment secondary to systemic vasculitis in EMC.
- This presentation differed from typical EMC-related cardiac events like myocardial infarction.
- No evidence of hypertension or renal involvement contributed to the heart failure.
Implications:
- Highlights the potential for EMC to manifest with primary cardiac dysfunction via microvascular compromise.
- Suggests a need for heightened awareness of cardiac microcirculation in EMC patients presenting with heart failure.
- Underscores the importance of considering vasculitis in unexplained ischemic heart failure.
- Emphasizes that EMC can cause heart failure through mechanisms beyond direct coronary artery occlusion.
Abstract:
The case of a 58-year old man affected by heart failure on ischemic basis, as clinical onset of essential mixed cryoglobulinemia (EMC) is reported. Laboratory assays, ECG at rest and exercise electrocardiogram, echocardiogram, myocardial scintigraphy, cardiac catheterization with coronarography, hepatic, bone and kidney biopsies confirmed the diagnosis. Cases of primitive heart involvement are rarely reported and are, usually, due to myocardial infarction. Nevertheless in the published series of cases, heart failure is often coupled with EMC and, not seldom, is the cause of death. In the present case heart failure was the dominant element of clinical symptomatology and wasn't consequent to renal involvement or hypertension, but was sustained by a myocardial contractile deficiency, on ischemic basis, with undamaged coronary circle at angiography. Therefore heart failure was ascribed to an involvement of coronary microcirculation in the course of systemic vasculitis.