Definition, diagnosis, treatment for scleroderma primary heart involvement.

Systemic sclerosis (SSc) is an autoimmune disease characterized by chronic inflammation leading to fibrosis in the skin and organs, including the heart. It is classified as SSc-primary heart involvement (pHI), defined as heart dysfunction primarily caused by SSc, excluding other causes. Several disease manifestations, including conduction abnormalities, cardiac arrythmias, myocarditis/pericardial involvement, heart failure (systolic and diastolic) and valvular dysfunction, are categorized within SSc-pHI. SSc-pHI has a high prevalence, high mortality, and increased risk for sudden cardiac death. Serum biomarkers, high sensitivity troponin and NT-proBNP, may help in screening, with electrocardiography, echocardiography, and Holter monitoring. Cardiac magnetic resonance imaging (cMRI) can confirm SSc-pHI and is considered gold standard for diagnosis, with limited use due to cost and availability. Treatment approaches are multifaceted depending on the manifestation of SSc-pHI but often require immunosuppression at a minimum. A multidisciplinary team including rheumatologists and cardiologists offers the best approach for diagnosis, management, and monitoring.