Breathlessness in systemic sclerosis - quantifying the contribution of cardiac fibrosis

Years funded:

Systemic sclerosis (often called scleroderma) is an autoimmune condition that causes fibrosis of the skin and internal organs.  Fibrosis (or scarring) of the heart muscle is an end-product of many conditions associated with heart muscle dysfunction and is associated with progressive heart failure, breathlessness, fatigue and markedly reduced survival. Given that fibrosis is a cardinal feature of systemic sclerosis, fibrosis of the heart muscle is likely to contribute significantly to disease progression and patient mortality. However, the prevalence, extent and contribution of fibrosis of the heart to patient ill-health have not been defined. 

Until now, heart fibrosis has been difficult to assess and there have not been any treatments for heart fibrosis. There are now well-validated means of imaging fibrosis of the heart with cardiac magnetic resonance imaging (CMR) and promising novel anti-fibrotic treatments are currently being trialled.

This study will use CMR to characterise the degree of heart fibrosis in a group of 40 patients with previously diagnosed systemic sclerosis.  Physical capacity and breathlessness will be assessed in patients by means of cardiopulmonary exercise testing. This quantification of physical limitation will then be related to the extent of heart fibrosis.  An association between these factors will enable us to test novel therapies in the future with a view to alleviating symptoms and improving patient survival.

Researcher Profile

Associate Professor Andre La Gerche

Institute: Baker Heart and Diabetes Institute
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