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Cardiovascular Manifestations of Systemic Sclerosis: An Overview of Pathophysiology, Screening Modalities, and
Kyu-In Lee1, Levy Jo Manuntag1, Alina Kifayat2
1From the Department of Medicine, Westchester Medical Center, Valhalla, NY.
Insights
Systemic sclerosis (SSc) is a complex disease with significant cardiovascular risks. Early screening and management of SSc-related heart conditions like pulmonary hypertension are crucial for improving patient outcomes.
Area of Science:
- Cardiology
- Rheumatology
- Systemic Sclerosis
Background:
- Systemic sclerosis (SSc), also known as scleroderma, is characterized by autoantibodies, vasculopathy, and fibroblast dysfunction.
- Cardiovascular manifestations are a major cause of mortality in SSc patients.
- Key cardiac issues include electrical conduction abnormalities, pulmonary hypertension, pericardial disease, and atherosclerosis.
Approach:
- A comprehensive literature review was conducted.
- The review focused on the cardiovascular manifestations of SSc.
- The analysis covered definitions, diagnostics, and treatment options for each cardiac complication.
Key Points:
- Early detection and management of cardiovascular complications in SSc are vital.
- Annual screening using noninvasive methods is recommended for SSc patients.
- Treatment strategies for SSc-related cardiac conditions align with general patient management based on clinical severity.
Conclusions:
- Cardiovascular disease significantly impacts mortality in systemic sclerosis.
- Proactive screening and timely intervention are essential for managing SSc's cardiac effects.
- Standard treatment protocols, adapted for SSc, are effective for managing cardiac complications.
Abstract:
Systemic sclerosis, previously known as scleroderma, is a heterogeneous, systemic disease that is defined by its 3 pathological hallmarks: the production of autoantibodies, small vessel vasculopathy, and fibroblast dysfunction, leading to an increased deposition of extracellular matrix. We conducted a review of the available literature that covers the cardiovascular manifestations of SSc: electrical conduction abnormalities, pulmonary hypertension, pericardial disease, and atherosclerosis. Within each major category, we will discuss the definition, diagnostics, and available treatment options. Increased mortality from cardiovascular complications necessitates early screening and management. Annual screening with noninvasive modalities is encouraged. The current management of each complication generally follows the management algorithms of patients regardless of SSc status and is dependent on the severity of the patient's clinical presentation.
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