Study finds subclinical issues in 25% of patients using 'more sensitive' tool by Steve Bryson, PhD | June 11, 2024

Problems with the heart’s left ventricle at a subclinical — not yet symptomatic and overt — level were evident in 1 in every 4 people with systemic sclerosis (SSc) taking part in an Australian study when a potentially more sensitive measure than left ventricle ejection fraction was used.

Both symptomatic or asymptomatic left ventricle dysfunction also associated with poorer physical function and survival, data showed.

These findings suggest “an under-appreciated burden of subtle LV [left ventricle] systolic dysfunction in SSc that has a significant impact on patient symptomatology,” the researchers wrote.

The study, “Prognostic and functional importance of both overt and subclinical left ventricular systolic dysfunction in systemic sclerosis,” was published in the journal Seminars in Arthritis and Rheumatism.

Left ventricle ejection fraction measures were normal in most study patients
SSc, also known as scleroderma, is marked by the abnormal buildup of scar tissue (fibrosis) in the skin and, potentially, other organs like the lungs, kidneys, heart, and those of the digestive system.

Heart involvement can be life-threatening in SSc, and advanced imaging techniques have shown scar tissue formation in heart muscle in up to 90% of patients without symptoms, the researchers noted. Asymptomatic myocarditis, or inflammation of the heart muscle, may also occur.

Despite these abnormalities, little is known about scleroderma-related dysfunction of the left ventricle, the side of the heart that pumps oxygenated blood throughout the body.

To address this gap, scientists across Australia collaborated to investigate the frequency of reduced left ventricle function in SSc.

A total of 1,141 patients were recruited from the Australian Scleroderma Cohort Study, a multicenter and observational disease study. All had at least one measure taken of left ventricle ejection fraction (LVEF), the percentage of blood pumped from the left ventricle with each heartbeat. Patients with a history of secondary or non-SSc causes of left ventricle dysfunction were excluded.

Nearly all participants (97.6 %) had a consistently normal LVEF of at least 50%, while 27 (2.4 %) had an LVEF of less than 50% at least once. Seven of them (0.6 %) had an LVEF of less than 40%.

Those with an LVEF of less than 50% were more likely to be male, to have diffuse cutaneous SSc, a larger left atria (the top left heart chamber), higher peak blood pressure in the right ventricle, and more frequent right ventricular dysfunction.
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These 27 patients also were more likely to have higher levels of inflammatory markers and to more often use medicines for heart-related issues and immunosuppression. Diseases that affected skeletal muscle, those attached to bones, also were more frequent in patients with an LVEF of less than 50%.

The self-reactive antibodies are an indicator of Sjögren’s syndrome by Patricia Inácio, PhD | May 21, 2024

Anti-SSA self-reactive antibodies, a hallmark of Sjögren’s syndrome, are associated with a higher risk of organ involvement in people with scleroderma, including the lungs and skin, a study in Japan shows.
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The findings suggest “anti-SSA may be a valuable biomarker of organ involvement and skin severity in patients with SSc [systemic sclerosis, also called scleroderma],” wrote the researchers, who said “periodic screening for these involvements should be recommended in patients with SSc” who are positive for such antibodies.

The study, “Clinical features of patients with systemic sclerosis positive for anti-SS-A antibody: a cohort study of 156 patients,” was published in Arthritis Research and Therapy.

Sjögren’s syndrome is a chronic inflammatory autoimmune disease wherein a misdirected immune attack affects the glands that produce tears and saliva. It’s called primary Sjögren’s syndrome when it appears as an isolated illness and secondary when it follows another autoimmune disease.

Anti-SSA antibodies are a diagnostic marker of Sjögren’s syndrome and are often detected in people with SSc. In fact, studies report that up to 24% of SSc patients have Sjögren’s syndrome.

While SSc patients may have anti-SSA antibodies, but not symptoms of Sjögren’s syndrome, “few studies have focused on patients with anti-SSA-positive SSc, and the clinical significance of this presentation is not fully understood,” wrote researchers in Japan who analyzed data from 156 patients with SSc who were followed at the Yokohama City University Hospital between 2018-2021.
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The patients were mostly (88.5%) women, were a median age of 69 and a mean disease duration of 13.3 years. As for SSc subtypes, most (75%) had limited cutaneous SSc and 25% had diffuse cutaneous SSc.

I share my story and hope it inspires someone to help in the fight
​by Sherlene Perkins | May 20, 2024

I love to have conversations with strangers and tell them about scleroderma.

I take time during brief encounters at the library, supermarket lines, the nail salon, and airport gates. The challenge is to provide the information in two minutes or less to spark and keep the listener’s attention. I ask if they are familiar with scleroderma and explain how it sneaks in and attacks parts of the body, causing arthritic pain, loss of fingers and toes, skin tightness, and the hardening of organs. I show them my hands and do the pinch test to demonstrate the thickness of my skin.

I also provide some general terminology while telling them that many patients diagnosed with scleroderma experience skin tightening, Raynaud’s phenomenon, and gastroesophagael reflux disease (GERD). Some may also have lung fibrosis, interstitial lung disease, and pulmonary hypertension. I make a point to mention hip-hop singer and actress Queen Latifah, and explain that scleroderma is what her mom died from. The late actor Bob Saget lost his sister to scleroderma as well.

During my chats, I mention the advocacy organizations I have been involved with that help rare disease patients maintain or improve their quality of life. These include the National Organization for Rare Disorders, Global Genes, the Rare Disease Legislative Advocates, the University of Michigan’s RENEW Scleroderma app, Northwestern Medicine, and Rare Patient Voice.
I will even direct people to my website.
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I encourage them to get involved with helping those less fortunate and explain we need access to better healthcare and other supportive services. I tell them that scleroderma is complicated. At times, the disease is silent, and someone won’t know what’s going on until new symptoms appear or an old one reappears.

Few vaccinated patients reported symptoms worsening, according to survey by Margarida Maia, PhD | June 4, 2024

More than half of people with systemic sclerosis (SSc) who hesitated to get vaccinated against COVID-19 were concerned they might have a flare-up, but very few vaccinated patients have reported their symptoms worsening, according to a 2021–2022 survey study.

“Even though the peak of the pandemic has passed, COVID-19 infections and hospitalizations continue to present substantial risk for vulnerable people with autoimmune rheumatic diseases, including SSc,” said the study’s researchers, who noted that patients who are unsure about a vaccination should be informed of its safety. The study, “COVID-19 vaccinations and infections among individuals with systemic sclerosis: a Scleroderma Patient-centered Intervention Network (SPIN) Cohort study,” was published in Seminars in Arthritis and Rheumatism.
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When vaccines against COVID-19 were made available, there was little information on their safety in people with autoimmune rheumatic diseases. Concerns that getting one may have serious side effects led many people to hesitate.