Scleroderma Patients at Higher Risk of Cardiovascular Diseases, Review Finds

People with scleroderma are at a higher risk of developing diseases that affect the heart or blood vessels — commonly known as cardiovascular diseases — compared with those who do not have the autoimmune disorder, a review study has found.

Physicians caring for scleroderma patients should keep this risk in mind and adopt preventive measures to avoid further organ deterioration and additional complications, the researchers said.

The review, “Systemic sclerosis and risk of cardiovascular disease A PRISMA-compliant systemic review and meta-analysis of cohort studies,” was published in the journal Medicine.

Scleroderma, also known as systemic sclerosis (SSc), is a chronic autoimmune disorder that affects the skin and multiple internal organs, including the heart. As the disease progresses, the skin becomes progressively thicker and internal organs increasingly scarred.

Although macrovascular disorders, such as stroke and heart attack (myocardial infarction), are not typically seen as a feature of scleroderma, studies have shown that many patients experience a series of alterations that are normally associated with these diseases. Macrovascular disorders are those that affect the larger blood vessels, including the aorta and the coronary arteries.

While other autoimmune diseases, including systemic lupus erythematosus and rheumatoid arthritis, have been linked with an increased risk of cardiovascular diseases, the association between these disorders and scleroderma is still unclear.

To better assess this possible relationship, researchers in China conducted a systematic review and meta-analysis, in which they searched the literature to gather data from studies comparing the risk of cardiovascular diseases in people with and without scleroderma. Notably, a meta-analysis is a statistical approach that pools data from multiple scientific studies.

Using four online literature search databases — Cochrane, Embase, Medline, and PubMed — the investigators identified a total of 2,608 potentially relevant studies published before Oct. 14, 2019.

After performing additional screenings, they selected seven studies involving 14,813 scleroderma patients and 5,506,836 individuals without the disease (controls) to be included in the final review and meta-analysis.

Two studies carried out in Europe and North America focused on the link between scleroderma and cardiovascular diseases. Pooled data from these studies, which involved a total of 3,185 patients, showed that, compared with controls, people with scleroderma were 2.36 times more likely to develop cardiovascular disorders.

In turn, two European studies investigated the association between scleroderma and peripheral vascular disease, a disorder in which blood vessels outside the heart start to narrow. People with scleroderma were 5.27 times more likely to develop peripheral vascular disease than were the controls, according to pooled data from these studies, which involved 3,636 scleroderma patients.

The link with heart attacks was evaluated in four of the seven studies — involving 6,210 people with scleroderma — included in the final meta-analysis. Again, the researchers found that scleroderma patients were at a higher risk than those without the disease. The analysis showed scleroderma patients were 2.36 times more likely to experience a heart attack than were controls.

Finally, the investigators analyzed data from five studies that focused on the link between scleroderma and stroke. These studies, which involved 10,649 patients, also indicated that those with scleroderma had a 1.52 times higher risk of having a stroke than people without scleroderma.

Despite the high degree of variability across the studies included in the analyses, most had a low risk of bias, meaning their findings were likely reliable.

Notably, none had data on standard cardiovascular risk factors, such as body mass index, which is a measure of adult body fat based on height and weight, smoking, and family history of cardiovascular disease.

“This meta-analysis revealed that SSc was associated with an increased risk of CVD [cardiovascular disease],” the researchers wrote.

“Future studies should focus on identifying risk factors for CVD in SSc, such as clinical subtypes or disease severity,” they added.

“Furthermore, clinicians who manage patients with [scleroderma] should be aware of the increased risk of CVD from the time of SSc diagnosis and undertake preventive measures,” the researchers concluded.