Inflammatory arthritis — a condition marked by joint inflammation that causes swelling, pain, and damage — affects about one-third of people with scleroderma, also known as systemic sclerosis (SSc), and is associated with worse health-related quality of life, or HRQoL for short, according to a new study from Australia.
“Our results reveal the significant impact that [inflammatory arthritis] has on patients, with the presence of [such inflammation] being negatively associated with employment, physical function, and HRQoL,” the researchers wrote. Given that such arthritis is treatable, “further research is needed to understand the extent of joint involvement in SSc and optimal strategies for its management,” the researchers wrote. Their study, “Inflammatory Arthritis in Systemic Sclerosis: Its Epidemiology, Associations, and Morbidity,” was published in the journal Arthritis Care & Research. Cognition is a problem for 50% of patients in study, while 29% were malnourished by Patricia Inácio, PhD | March 5, 2024 Cognitive difficulties affected half of all scleroderma patients in a recent study in Italy, while malnutrition was found in more than one quarter.
While no correlation was seen between cognitive impairment and malnutrition in the 100 patients involved, the presence of these complications associated with greater functional disability and a poorer quality of life. Identifying them early is “pivotal to better address the chronic needs of patients affected by this disease,” researchers wrote in the study, “Role of cognitive impairment and malnutrition as determinants of quality of life in patients with systemic sclerosis,” published in the Journal of Scleroderma and Related Disorders. The condition is associated with inflammation, end-organ involvement by Patricia Inácio, PhD | February 27, 2024 Muscle disease is common in people with scleroderma and is associated with inflammation and specific end-organ involvement, according to an Australian study.
The findings underscore the “clinical, functional, and prognostic importance of simple biomarkers ” for identifying SSc-related muscle disease, the study’s researchers wrote in “Proximal weakness and creatine kinase elevation in systemic sclerosis: clinical correlates, prognosis and functional implications,” which was published in Seminars in Arthritis and Rheumatism. Scleroderma, also called systemic sclerosis (SSc), is a chronic disease that features excessive scarring of the skin and other organs due to abnormal immune responses against the body’s own tissues. Muscle disease, or myopathy, can be common in SSc and develop due to the indirect effects of the disease, such as malnutrition. A previous study using MRI scans showed muscle involvement was found in up to 40% of asymptomatic SSc patients. A lack of consensus remains about what SSc-myopathy is and of biomarkers for muscle disease in SSc, leading researchers to assess whether two routine clinical biomarkers of muscle disease — elevated blood levels of creatine kinase (CK) and proximal muscle weakness (PW) — could help identify those at risk for more severe outcomes. The scientists analyzed data from patients enrolled in the Australian Scleroderma Cohort Study between 2007 and 2023 who had at least one assessment of PW and CK levels during follow-up. Oral therapy shown to safely improve lung function in Phase 2 trial by Andrea Lobo, PhD | February 20, 2024 Certa Therapeutics’ FT011, an investigational oral therapy for systemic sclerosis (SSc), has been granted fast track designation by the U.S. Food and Drug Administration (FDA).
It comes following Phase 2 clinical trial data that showed that FT011 was safe and well tolerated, and led to clinically meaningful improvements in lung function for some participants. The therapy also helped to ease physical disability for more than half of the patients in the small study. The FDA fast track designation, which provides incentives such as more frequent meetings with the regulatory agency and potential eligibility for priority review and accelerated approval, is meant to speed the development of treatments for serious or life-threatening conditions. “We are thrilled to have received fast track designation which supports further acceleration of the FT011 clinical development program,” Darren Kelly, PhD, Certa’s founder and CEO, said in a company press release. Kelly added that the designation “also provides validation of FT011’s potential to offer patients with scleroderma the first anti-fibrotic and disease modifying treatment of this type.” In my debut column, I share the hurdles I face in my life as a warrior by Sherlene Perkins | February 19, 2024 Yes, I’m a warrior. I was classified as one long before my scleroderma diagnosis since I’m an African American woman and single mom of two adult children. God has been preparing me to be a great warrior since birth.
Learning that I had scleroderma was a relief. The symptoms started July 2019 after six months of excruciating pain and an inability to use my hands. Amid the diagnosis and symptoms, I found solace in thinking sardonically, “OK. Now what?” The shock was that the doctors told me there is no cure. They prescribed prednisone to help with inflammation and methotrexate to suppress my immune system. Disbelief about my bad luck filled me. Now what? 775 participants from SPIN were asked to complete UCLA Loneliness Scale-6by Steve Bryson, PhD | February 13, 2024 More conversations, either one on one or in groups via telephone or virtually, along with in-person household activities and conversations, were significantly associated with less loneliness during the COVID-19 pandemic among people with systemic sclerosis (SSc), a study indicates.
Conversations and activities at the beginning of the pandemic didn’t predict loneliness over the following two years, however. The findings suggest “sustained levels of interactions over time were potentially more useful than levels of interactions when the pandemic began,” the researchers wrote in “Moderators of Loneliness Trajectories in People with Systemic Sclerosis During the COVID-19 Pandemic: A SPIN COVID-19 Cohort Longitudinal Study,” which was published in the International Journal of Behavioral Medicine. SSc, or scleroderma, is an autoimmune disorder that affects connective tissue, damaging the skin and possibly the internal organs, including the heart and blood vessels, lungs, stomach, and kidneys. During the COVID-19 pandemic, people with SSc had a higher risk of disease and death due to compromised lung function, overall poor health, and the use of immunosuppressant medications. As a result, many faced long periods of isolation, which may have been worsened by physical limitations, such as fatigue and chronic pain. Such isolation may have induced feelings of loneliness, an unpleasant emotional response often associated with a perceived lack of social relationships that fails to fulfill social needs. A Phase 2 trial will explore effect of AM1476 on skin thickness, lung function by Andrea Lobo, PhD | February 6, 2024 AM1476, AnaMar’s investigational anti-fibrotic medication, has been granted orphan drug status by the U.S. Food and Drug Administration (FDA) and the European Medicines Agency (EMA) for treating scleroderma.
To get this designation, a medication must be intended to treat a life-threatening rare disease that affects fewer than five in 100,000 people in Europe or under 200,000 people in the U.S. The designation provides certain incentives, including regulatory fee reductions and the potential for extensive market exclusivity — seven years in the U.S. and 10 in the European Union — if approved. The company plans a Phase 2 clinical trial to evaluate the treatment in people with scleroderma and interstitial lung disease (ILD), a group of diseases marked by lung scarring. The study will evaluate AM1476’s effects on skin thickness and lung function in 60 patients with diffuse cutaneous scleroderma and ILD over a year. “This is a significant milestone and underscores the significant unmet need for novel medicines to prevent, heal, and slow organ scarring from fibrotic diseases, which are often progressive and can have a poor prognosis,” Ulf Ljungberg, PhD, AnaMar’s CEO, said in a company press release. Scleroderma, also known as systemic sclerosis, is marked by inflammation and fibrosis, or uncontrolled tissue hardening and scarring, in the skin and even the heart, kidneys, and lungs. Up to 80% of people with SSc may develop ILD, which leads to reduced lung function and breathing problems. Spike promotes infiltration of pro-inflammatory cells in mouse model by Andrea Lobo, PhD | January 30, 2024 SARS-CoV-2, the virus that causes COVID-19, may accelerate the development and progression of systemic sclerosis (SSc) through fibrosis (scarring), inflammation, autoantibody production, and blood vessel damage.
That’s according to a study in South Korea that shows the SARS-CoV-2 spike protein that’s required for the virus to infect human cells promotes the infiltration of pro-inflammatory cells in the skin and lung tissue of an SSc mouse model. The study, “SARS-CoV-2 spike protein accelerates systemic sclerosis by increasing inflammatory cytokines, Th17 cells, and fibrosis,” was published in the Journal of Inflammation. SSc is an autoimmune disease that features scar tissue accumulating in the skin and potentially the internal organs, including the heart, kidney, lungs, and digestive tract. The disease is thought to develop due to excessive scarring, autoantibodies being produced that mistakenly attack healthy tissues, and damage to small blood vessels. COVID-19 induces the overactivation of immune cells, inflammation, autoantibody production, and bleeding disorders, similar to mechanisms in SSc and other autoimmune diseases. “While there is a single case report suggesting an association between COVID-19 and SSc, the effects of COVID-19 on SSc are not yet fully understood,” wrote the researchers who used human embryonic kidney cells that were manipulated to produce the SARS-CoV-2 spike protein and the ACE2 receptor, a protein to which the virus binds to enter cells. The spike protein is also known to induce fibrosis, the investigators said. Its presence significantly increased the activity of proteins associated with fibrosis, namely alpha-SMA and Col1a1. Patients in interview study talk of the various life changes they have to confront by Marisa Wexler, MS | January 23, 2024 Living with scleroderma requires adapting to changes in a woman’s sense of self, but practicing gratitude and accepting change can help patients reclaim themselves, a study aiming for a “grounded theory” of identify management reports.
Its scientists said these findings may lay the groundwork for future studies aiming to develop interventions to help people with scleroderma hold on to their sense of self while navigating life with the progressive disease. The study, “Process of Maintaining Self in Individuals Living With Systemic Sclerosis: A Grounded Theory Study of American Women,” was published in the Western Journal of Nursing Research. Flow-mediated dilation 'helpful tool in the overall assessment of vascular injury' by Lindsey Shapiro, PhD | January 16, 2024 A non-invasive technique called flow-mediated dilation (FMD) could be useful for monitoring blood vessel alterations in people with systemic sclerosis (SSc) and predicting the likelihood of certain blood flow (vascular) complications, a study has found.
The test findings show distinct differences between SSc patients and healthy people, and were more altered in SSc patients who had been living with the disease longer and had certain vascular symptoms, including pulmonary arterial hypertension (PAH). This correlated well with the degree of damage seen in other tests of vascular damage as well as blood biomarkers of the endothelial cell dysfunction thought to drive blood vessel changes in SSc. The study, “Flow Mediated Dilation in Systemic Sclerosis: Association with clinical findings, capillaroscopic patterns and endothelial circulating markers,” was published in Vascular Pharmacology. Endothelial dysfunction drives blood vessel damage, blood flow changes Alterations to the endothelial cells that line blood vessels — called endothelial dysfunction — drive blood vessel damage and blood flow changes in SSc. As a result, patients may experience certain vascular complications such as Raynaud’s phenomenon, PAH, or digital ulcers. Small blood vessels (i.e. capillaries) as well as larger vessels (i.e. veins and arteries) in patients may be affected, known as microvascular and macrovascular damage, respectively. Microvascular damage is more common and better characterized. These changes can be assessed using a technique called nailfold video-capillaroscopy (NVC), where the capillaries under the skin near the fingernail beds are visualized under a microscope. A method for detecting macrovascular damage is not as well established. FMD, which looks at an artery’s diameter in response to blood flow restriction, could be one way to do this, but it is not yet widely used in clinical practice. |
AuthorScleroderma Queensland Support Group Archives
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