Even minor changes in heart function are associated with significant changes to mortality risk.
There is a growing awareness of what scleroderma heart involvement is and how to diagnose it – and while one expert feels the guidelines in this space aren’t perfect, they’re better than nothing.
Speaking as part of a concurrent session on systemic sclerosis at the recent ARA ASM in Adelaide, Dr Laura Ross, a Melbourne-based rheumatologist, presented on scleroderma heart involvement – a less common, but no less important, manifestation of disease.
“It has been documented since the 1940s or 1950s that scleroderma has direct effects on the heart,” Dr Ross explained.
“There are a number of histopathological studies that really show there is evidence of fibrosis, inflammation and vasculopathy within the hearts of patients with scleroderma – the classical pathogenic triad that we all refer to when we think about what causes scleroderma.”
Large cohort studies suggest that 5-7% of scleroderma patients have clinically relevant heart involvement. But the rarity of scleroderma heart involvement does not mean it is without serious consequences, as Dr Ross pointed out.
“In an elegant study performed by Dr Jenny Hao, a colleague of mine at St Vincent’s, looking at an international inception cohort of patients with scleroderma, myocardial involvement from scleroderma is the third leading cause of death – behind interstitial lung disease and pulmonary arterial hypertension… suggesting that this is a very lethal manifestation of scleroderma and something that we should take very seriously,” she told delegates.
And it doesn’t take much of a decrease in heart function to lead to poor outcomes. Data from Dr Ross’ PhD student Dr Jessica Fairley, indicated that even a “mild” reduction of in left ventricular ejection fraction – that is, less than 50%, “which cardiologists would consider to be almost not worth remarking on”, according to Dr Ross – was associated with a 2.3-fold increase in the likelihood of death.
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“Scleroderma heart involvement has significant implications in terms of poor prognosis, but also in terms of symptoms that patients experience,” said Dr Ross.
So, given the risks associated with scleroderma heart involvement, how does one define it?
An international group of and clinicians operating under the auspices of the World Scleroderma Foundation and the European Society of Cardiology’s Heart Failure Association proposed the following definition in 2022:
“Systemic sclerosis primary heart involvement comprises cardiac abnormalities that are predominantly attributable to systemic sclerosis rather than other causes or complications,” where the list of other cardiac conditions includes ischaemic heart disease, arterial hypertension and interstitial lung disease.
Dr Ross felt there was plenty of room for improvement in the initial definition, which was created by consensus following a systematic literature review.
“I think one of the problems with this definition is that scleroderma heart involvement still remains a diagnosis of exclusion,” she told delegates
“But at least we have an initial definition, and this is something that hopefully progress in the future to make it more of a positive diagnosis.”
A selection of authors involved in the definition work has also advocated for better screening and management of patients with scleroderma heart involvement – including an algorithm that groups patients based on their potential risk, and where the extent of screening varies based on this potential risk.
Screening approaches used in the diagnostic algorithm include excluding secondary causes of heart disease by exploring signs and symptoms, understanding disease characteristics and biomarkers through ECG and blood tests and more invasive approaches including endomyocardial biopsy and electrophysiological studies.
“It’s also important to note that the guidelines suggest that patients should be screened within a multidisciplinary team – and I think that this requires some further thought,” noted Dr Ross.
“The field of cardiorheumatology is a very new and emerging subspecialty of cardiology. These authors don’t propose what the optimum multidisciplinary team might look like, and I think it’s an area of future research to see how we could work more closely with our cardiology colleagues to develop rigorous screening algorithms and comprehensive multidisciplinary care models for our patients.”
Dr Ross cautioned against the use of routine and universal screening, citing local data suggesting that all patients with scleroderma heart involvement will have some level of abnormality on a cardiac MRI scan.
“I don’t think the evidence is there to support the treatment of isolated cardiac MRI abnormalities,” she said.
“But as the evidence base in this field evolves, potentially we can screen for heart involvement similar to the way we currently do routine screens for pulmonary vascular disease.”
When considering all the available evidence, Dr Ross highlighted that an important first step to optimising cardiac outcomes in patients with scleroderma heart involvement is keeping it front of mind.
“It’s a difficult diagnosis to make, but we’re not going to make it if we don’t think about it,” she concluded.
ARA 25 was held at the Adelaide Convention Centre from 3-6 May.
