Primary myocardial disease in scleroderma-a comprehensive review of the literature to inform the UK Systemic Sclerosis Study Group cardiac working group

Rheumatology (Oxford). 2017 Jun 1;56(6):882-895. doi: 10.1093/rheumatology/kew364.

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.

Keywords: SSc-cardiomyopathy; cardiac disease; primary myocardial disease; scleroderma; systemic sclerosis.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Adult
  • Age of Onset
  • Aged
  • Aged, 80 and over
  • Arrhythmias, Cardiac / diagnosis
  • Cardiomyopathies / diagnosis*
  • Cardiomyopathies / mortality
  • Child
  • Diagnosis, Differential
  • Echocardiography / methods
  • Electrocardiography / methods
  • Female
  • Humans
  • Magnetic Resonance Angiography
  • Male
  • Middle Aged
  • Prognosis
  • Quality of Life
  • Scleroderma, Systemic / diagnosis*
  • Scleroderma, Systemic / mortality
  • Severity of Illness Index
  • Sex Factors
  • Young Adult