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Published on: June 16, 2020
Acute Heart Failure in Advanced Systemic Sclerosis With Multiorgan Involvement
Hanna Maria Eivindson1, Esben Uggerby Naesser2, Per Ivarsen3
1Department of Cardiology, Aarhus University Hospital, Aarhus, Denmark, auh.dk.
Heart failure is rarely caused by autoimmune diseases. This case highlights diffuse cutaneous systemic sclerosis (dcSSc) presenting initially with severe heart failure, emphasizing its potential for significant cardiac morbidity.
Area of Science:
- Cardiology
- Rheumatology
- Internal Medicine
Background:
- Heart failure is infrequently associated with autoimmune conditions.
- Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by microvascular damage and fibrosis.
- Diffuse cutaneous systemic sclerosis (dcSSc) involves widespread skin and internal organ manifestations.
Purpose of the Study:
- To report a rare case of dcSSc presenting initially with severe heart failure.
- To underscore the importance of considering cardiac involvement in SSc.
- To highlight the diagnostic challenges and clinical significance of primary cardiac manifestations in dcSSc.
Main Methods:
- A case report of a male patient in his 60s with no prior cardiovascular risk factors.
- Clinical presentation included dyspnea, pulmonary edema, cyanosis, ischemic ulcers, dysphagia, and weight loss.
- Diagnostic workup involved echocardiography, computed tomography, and confirmation of dcSSc.
Main Results:
- The patient presented with severe left ventricular dysfunction (ejection fraction of 10%) and cardiogenic pulmonary edema.
- Imaging studies ruled out malignancy but confirmed pulmonary edema and pleural effusion.
- The patient was diagnosed with diffuse cutaneous systemic sclerosis (dcSSc) as the underlying cause.
Conclusions:
- Primary cardiac involvement as an initial presentation of dcSSc is uncommon.
- Cardiac complications in dcSSc can lead to significant morbidity and mortality.
- Early recognition and management of cardiac dysfunction are crucial in patients with dcSSc.
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