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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Bissell, Lesley-Anne, Yusof, Md Yuzaiful Md, and Buch, Maya H.
- Subjects
TREATMENT of cardiomyopathies ,AGE distribution ,AGE factors in disease ,ARRHYTHMIA ,BLACK people ,CORONARY disease ,CUTANEOUS manifestations of general diseases ,DIFFERENTIAL diagnosis ,HEART failure ,MEDICAL databases ,INFORMATION storage & retrieval systems ,MEDICAL information storage & retrieval systems ,MEDLINE ,CARDIOMYOPATHIES ,QUALITY of life ,RACE ,SYSTEMIC scleroderma ,PHENOTYPES ,SYSTEMATIC reviews ,EVIDENCE-based medicine ,DISEASE complications ,PROGNOSIS - 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. [ABSTRACT FROM AUTHOR]
- Published
- 2017
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