Younger Age, Thicker Skin at Scleroderma Onset Increase Risk of Heart Muscle Inflammation, Study Suggests

People with scleroderma who were younger and had thicker skin at the onset of their disease experience an increased risk of heart muscle (myocardial) inflammation, a new study suggests.

The research, “Clinical and laboratory predictions of myocardial inflammation as detected by cardiac magnetic resonance imaging in patients with systemic sclerosis: A pilot study,” was published in the International Journal of Rheumatic Diseases.

Cardiac involvement in scleroderma is associated with pulmonary arterial hypertension and kidney dysfunction, and can affect the myocardium (the heart muscle layer). The hallmark of myocardial involvement in these patients is inflammation and fibrosis, or scarring. Myocardial inflammation is common in early-onset scleroderma, so early detection and treatment may help prevent fibrosis and adverse outcomes.

The gold standard for diagnosing myocardial inflammation is endomyocardial biopsy, which is an invasive procedure with low sensitivity that can result in serious complications.

Cardiac magnetic resonance imaging (MRI) is non-invasive and highly reliable, and can be used to detect structural and functional alterations of the heart. Also, it may differentiate inflammation from fibrosis in the myocardium.

Researchers at the Scleroderma Clinic at Khon Kaen University, in Thailand, hypothesized that myocardial inflammation detected with cardiac MRI would correlate with the severity of skin thickness and with levels of inflammatory markers and cardiac enzymes in people with scleroderma.

The study included a total of 30 Thai adults with scleroderma (mean age of 57 years, 19 women), all with disease onset within the last four years. All underwent cardiac MRI, and clinical and laboratory assessments on the same date.

Most participants (73.3%) had diffuse cutaneous scleroderma. Median duration of disease was two years.

Results revealed myocardial inflammation in 22 patients (73.3%). A statistical analysis revealed that every five years of increased age at scleroderma onset correlated with a more than two-fold lower risk of myocardial inflammation.

In addition, every five‐point increase in the modified Rodnan skin score (mRSS) of skin thickness at disease onset was associated with a 2.64-fold greater risk for myocardial inflammation.

“An increased risk of myocardial inflammation was associated with young age and high mRSS at onset,” the researchers wrote.

However, myocardial inflammation was not associated with scleroderma subtype, internal organ involvement, inflammatory markers (such as C-reactive protein and erythrocyte sedimentation rate), and cardiac and muscle enzymes in this patient population.

This suggests “that inflammatory markers and/or cardiac enzymes cannot be used as a guide for identifying myocardial inflammation in [scleroderma] patients, so it cannot replace cardiac MRI,” the team said.

“A longitudinal study of the myocardial inflammation along with treatment and clinical outcome monitoring is fundamental for better care and improved survival of [scleroderma] patients,” the scientists said.