1. Primary myocardial disease in scleroderma—a comprehensive review of the literature to inform the UK Systemic Sclerosis Study Group cardiac working group
- Author
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Yuzaiful Md Yusof, Lesley-Anne Bissell, and Maya H Buch
- Subjects
Male ,Cardiomyopathy ,Disease ,030204 cardiovascular system & hematology ,Systemic scleroderma ,Severity of Illness Index ,Scleroderma ,Electrocardiography ,0302 clinical medicine ,Pharmacology (medical) ,Age of Onset ,Child ,skin and connective tissue diseases ,Aged, 80 and over ,integumentary system ,Arrhythmias, Cardiac/diagnosis ,Middle Aged ,Prognosis ,Echocardiography ,Cardiology ,Female ,Cardiomyopathies ,Adult ,medicine.medical_specialty ,Adolescent ,Electrocardiography/methods ,Echocardiography/methods ,Cardiomyopathies/diagnosis ,Diagnosis, Differential ,Young Adult ,03 medical and health sciences ,Sex Factors ,Rheumatology ,Internal medicine ,Severity of illness ,medicine ,Humans ,Intensive care medicine ,Aged ,030203 arthritis & rheumatology ,Scleroderma, Systemic ,business.industry ,Arrhythmias, Cardiac ,medicine.disease ,Scleroderma, Systemic/diagnosis ,Heart failure ,Quality of Life ,Differential diagnosis ,Age of onset ,business ,Magnetic Resonance Angiography - Abstract
Cardiac disease is prevalent in SSc and associated with a poor prognosis. Differentiating primary myocardial disease (SSc-cardiomyopathy) from ischaemic heart disease is difficult and the disease phenotype most at risk is unclear. A comprehensive literature review was performed to inform the UK Systemic Sclerosis Study Group for cardiac disease tasked with producing a best practice pathway for the management of cardiac disease in SSc. This review describes the prevalence of SSc-cardiomyopathy, its associated greater mortality and various manifestations (e.g. heart failure, arrhythmias and diastolic dysfunction). The limited evidence suggests SSc-cardiomyopathy is associated with other poor prognostic indicators, including diffuse cutaneous disease, positive SSc-specific serology, black ethnicity, older age at disease onset, tendon friction rubs, abnormal nail-fold capillaroscopy and worse quality-of-life scores. Differentiating SSc-cardiomyopathy from ischaemic heart disease requires well-planned studies. Non-invasive investigative techniques are improving the understanding of its pathophysiological basis.
- Published
- 2016