Definition, diagnosis, treatment for scleroderma primary heart involvement

Area of Science:

• Cardiology
• Rheumatology
• Immunology

Background:

• Systemic sclerosis (SSc) is an autoimmune disease causing inflammation and fibrosis.
• Primary heart involvement (pHI) in SSc includes various cardiac dysfunctions.
• SSc-pHI is associated with high mortality and sudden cardiac death risk.

Purpose of the Study:

• To summarize the characteristics, diagnosis, and management of SSc-primary heart involvement.
• To highlight the importance of early detection and multidisciplinary care for SSc-pHI.

Main Methods:

• Review of SSc-pHI manifestations, diagnostic tools, and treatment strategies.
• Emphasis on serum biomarkers (troponin, NT-proBNP), ECG, echocardiography, Holter monitoring, and cardiac MRI.
• Discussion of multidisciplinary team approach involving rheumatologists and cardiologists.

Main Results:

• SSc-pHI encompasses conduction abnormalities, arrhythmias, myocarditis, heart failure, and valvular dysfunction.
• Biomarkers and imaging techniques aid in screening and diagnosis, with cMRI as the gold standard.
• Treatment is multifaceted, often requiring immunosuppression.

Conclusions:

• SSc-pHI is a serious complication of systemic sclerosis requiring prompt diagnosis and management.
• A collaborative approach integrating rheumatology and cardiology is essential for optimal patient outcomes.
• Continued research into biomarkers and targeted therapies is needed.