Protein may serve as tool for evaluating long-term dialysis risk, said researchers by Katherine Poinsatte | January 21, 2025

​Scleroderma (SSc) patients who’ve had a scleroderma renal crisis, a life-threatening complication of SSc, have higher levels of placental growth factor (PIGF), protein that stimulates blood vessel growth, in their blood than those who haven’t had one, a study in France shows.

Among French patients who’d had a scleroderma renal crisis, higher PIGF levels were also found in those who reached end-stage kidney failure compared with SSc patients who didn’t need dialysis, which is a treatment to clean the blood.

“Serum [blood] PlGF may identify the risk of [scleroderma renal crisis] occurrence among SSc patients with a good specificity and represents a potential tool for long-term dialysis risk evaluation,” the researchers wrote. The study, “Pilot Study of diagnostic performances of vascular biomarkers soluble fms-like tyrosine kinase and placental growth factor in scleroderma renal crisis,” was published in Kidney International Reports. 
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In a scleroderma renal crisis, abrupt blood pressure changes and acute kidney failure augurs a risk of of chronic kidney disease and dialysis. One of the few clinically measurable risk factors for developing scleroderma renal crisis is the presence of antibodies against RNA polymerase III. No biomarker has been able to help predict kidney prognosis after a scleroderma renal crisis, however.
PIGF has been found at higher levels in SSc patients with pulmonary hypertension, a condition where blood pressure in the lungs is abnormally high, compared with people with SSc but without pulmonary hypertension.

Classifying disease by skin symptoms was not associated with either by Katherine Poinsatte | January 14, 2025

Organ involvement and outcomes among people with systemic sclerosis (SSc) are strongly associated with seven specific self-reactive antibodies, a French study has found.

Among their findings, researchers identified antibodies that targets RNA polymerase III as strongly associated with scleroderma renal crisis, a rare and severe manifestation of SSc marked by high blood pressure, and kidney and heart failure.   Classification by skin symptoms wasn’t associated with survival or organ involvement.

“The systematic and accurate determination of [specific self-reactive antibodies] at diagnosis could help clinicians to better stratify the individual risk of developing SSc complications and personalize monitoring,” the researchers wrote. The study, “Impact of autoantibody status on stratifying the risk of organ involvement and mortality in SSc: experience from a multicentre French cohort of 1605 patients,” was published in RMD Open. 

SSc, also called systemic sclerosis, features excessive scarring in the skin and may also occur in the heart, kidney, lungs, and gastrointestinal tract. Patients are divided into two main subtypes, limited and diffuse, based on the extent of their skin symptoms. Limited SSc patients may have skin symptoms on the face, arms, hands, and fingers, while people with diffuse SSc have widespread skin fibrosis, or scarring, and a higher risk of internal organ involvement.

The skin-based subtypes have traditionally predicted organ involvement and mortality. However, given that not all SSc patients exhibit skin fibrosis and that serious lung involvement may occur in both subtypes, the researchers believe better ways of predicting organ involvement and disease outcomes are needed.

“The skin phenotype is not a sufficient and reliable factor to formally predict organ damage and disease complications for clinicians,” the researchers wrote.
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The immune system of people with SSc makes self-reactive antibodies that mistakenly attack healthy tissue. While several specific autoantibodies have been linked to the disease and particular organ involvement, the exact biological consequences of self-reactive antibodies are poorly understood.

Dual-pathway treatment aims for greater efficacy, benefit
by Andrea Lobo, PhD | January 7, 2025

​Zura Bio has launched a Phase 2 trial to evaluate the safety and efficacy of tibulizumab, its experimental dual pathway antibody to treat systemic sclerosis (SSc).

The TibuSURE trial is expected to enroll as many as 80 adults with early diffuse cutaneous SSc. They will receive tibulizumab or a placebo for 24 weeks (about six months). This will be followed by an open-label extension of 28 weeks, in which all participants will receive the treatment.

“We believe tibulizumab’s dual-pathway approach holds the potential to be best-in-class, aiming to provide deeper efficacy and greater benefits for patients affected by this life-threatening autoimmune disease,” Kiran Nistala, PhD, Zura Bio’s chief medical officer and head of development, said in a company press release. “The initiation of the TibuSURE study marks a significant milestone in addressing certain urgent, unmet needs of this patient population and advancing our mission to improve the lives of those affected by autoimmune and inflammatory conditions.”

SSc is an autoimmune disease characterized by inflammation and fibrosis, or the accumulation of scar tissue in the skin and internal organs, such as the lungs, heart, kidneys, and digestive tract.
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Two therapies (Ofev and Actemra) are available to treat severe lung complications associated with the disease, but there is no approved treatment for multiple organ manifestations.

GI symptoms are my constant unwanted companion by Tomisa Starr | December 18, 2024

​I think of scleroderma as my constant companion — one that tries to keep me from having a life of my own. Whenever I want to do anything outside of my home or lead a productive life, scleroderma always seems to hold me back. It’s like the friend I can’t take anywhere because they’re always doing things to embarrass me.

Of all the problems caused by my scleroderma, gastrointestinal (GI) issues are the worst. These are my most embarrassing and painful symptoms.
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Stomach noises may be embarrassing, but they’re a sign that the GI tract is working as it should. These sounds mean that the food we’ve eaten is being propelled through our digestive system and is on its way to the intestines. In the past, I’d occasionally hear my stomach churning whenever I was especially hungry, and I could hear my intestines working to expel gas from my body.
If there’s anything worse than the dismay most people experience when their stomach makes embarrassing noises, it’s when those noises suddenly stop and you realize that something is wrong.