Study probes autoimmunity link between SSc and primary biliary cholangitis by Andrea Lobo, PhD Systemic sclerosis (SSc) patients with primary biliary cholangitis (PBC), a disease that causes inflammation and scarring of the liver bile ducts, have milder symptoms that rarely lead to major complications, compared to patients with SSc alone.
A study with that finding also determined SSc-only patients had a higher incidence of heart and pulmonary diseases at follow-up, as well as new digital ulcers, than those with SSc and PBC. Autoimmunity may be a link between SSc and PBC, and patients with both conditions “may be considered a real crossroad of polyautoimmunity that seem to alleviate the SSc phenotype [characteristics],” the researchers wrote. The study, “Systemic sclerosis and primary biliary cholangitis: Longitudinal data to determine the outcomes,” was published in the Journal of Scleroderma and Related Disorders. SSc is caused by activation of the immune system, leading to inflammation and accumulation of scar tissue (fibrosis) in the skin and internal organs. Also called scleroderma, SSc has been associated with other autoimmune diseases, such as PBC. PBC-specific autoantibodies — meaning those attacking the body’s own tissues — have been associated with higher mortality. The prevalence of PBC in SSc patients is estimated to be 2-3% higher than in the general population. But PBC-specific autoantibodies are even more frequent. The condition is more frequent in people with limited SSc, characterized by skin symptoms in the face, arms, hands, and fingers, and has been suggested to identify SSc patients with milder body-wide disease. Elevated levels tied to more severe interstitial lung disease by Patricia Inácio, PhD Elevated blood levels of the cold-inducible RNA-binding protein (CIRP) are linked with more severe interstitial lung disease (ILD) in systemic sclerosis (SSc) patients and may be a marker of disease activity, a study from Japan reports.
“Our results suggest that serum [blood] CIRP levels may serve as a useful serological marker of SSc-ILD in terms of disease activity and therapeutic effects,” the researchers wrote. These findings “hold the potential to pave the way for a novel design for the treatment of patients with SSc-ILD.” The study, “Serum cold-inducible RNA-binding protein levels as a potential biomarker for systemic sclerosis-associated interstitial lung disease,” was published in the journal Scientific Reports Protein’s levels a potential blood marker of SSc-ILD progression ILD comprises a group of lung disorders marked by inflammation and fibrosis (scarring) that causes lung tissue to thicken and stiffen, restricting the volume of air that enters and leaves the lungs. While blood biomarkers have been proposed, “few of them could help estimate the progression of SSc-ILD,” the researchers wrote. Cells in damaged tissues release warning signaling molecules — called damage-associated molecular patterns (DAMPs) — as a way of alerting and activating the immune system. Several DAMPs have been reported to be involved in SSc. The CIRP protein, recently identified as a DAMP, has rising levels following exposure to stressors such as low oxygen levels, UV radiation, and glucose (sugar) deprivation. CIRP, a “general stress-response protein,” is found in several tissues, including the skin, lungs, and heart. While the protein recently was implicated in the development of idiopathic pulmonary fibrosis, its role in SSc is not well known. Findings: Education, marital status may affect symptoms by Margarida Maia, PhD Having a higher level of education or being married may mean fewer symptoms of depression for people with systemic sclerosis (SSc), according to a systematic review of multiple studies.
Researchers also observed that lung involvement, breathing problems, and tender joints were linked to more symptoms of depression, but not anxiety. Because there was not much evidence available, “future studies should examine prevalence of mood and anxiety disorders in SSc in large, representative samples,” the researchers wrote. The systematic review, “Results from a living systematic review of the prevalence of mood and anxiety disorders and factors associated with symptoms in systemic sclerosis,” was published in the journal Scientific Reports. People with SSc experience a wide range of symptoms caused by a buildup of scar tissue in the skin and possibly internal organs such as the heart, lungs, kidneys, and intestinal tract. “Disease presentation is extremely heterogeneous, and its course is unpredictable,” the researchers wrote. Because they are unsure of how their disease will progress, many people with SSc experience changes in mood or begin to feel anxious. Also contributing to mood and anxiety disorders are “high levels of chronic pain, fatigue, body-image distress, overall disability, increased risk of mortality, and limited treatment options,” the scientists added. Patients positive for Scl-70 antibodies at more risk of major organ involvement by Steve Bryson, PhD People with systemic sclerosis (SSc) who test positive for Scl-70 self-reactive antibodies are at a higher risk of major organ involvement, particularly the lungs, than those with other types of autoantibodies, a study suggested.
Digestive tract involvement was associated with the presence of anti-nuclear (ANA) autoantibodies. SSc patients with Scl-70 autoantibodies should be monitored for lung disease, regardless of whether their disease is limited to the skin or occurs in both skin and internal organs, the researchers recommended. “Understanding these risk factors might help with earlier diagnosis and better disease management for people with SSc,” they noted in the autoantibody study, “Clinical phenotype in scleroderma patients based on autoantibodies,” which was published in Rheumatology Advances in Practice. SSc, also called scleroderma, is an autoimmune disease that features the buildup of scar tissue in the skin and several other organs, potentially. It’s usually classified into two subtypes, based on the extent of skin symptoms. Limited cutaneous SSc (lcSSc) is indicated by skin symptoms confined to the face and arms below the elbows. Diffuse cutaneous SSc (dcSSc) involves widespread skin scarring that’s accompanied by internal organ damage. “However, dividing SSc according to skin involvement might be too simplistic, meaning that we could overlook a wider spectrum of the disease,” the researchers wrote. People with SSc have self-reactive autoantibodies that mistakenly target cellular components in their own tissues, including Scl-70, ANA, and anti-centromere (ACA) autoantibodies. While lcSSc has been associated with anti-ACA autoantibodies and dcSSc with anti-Scl-70 autoantibodies, some people with lcSSc are positive for Scl-70 autoantibodies and some with dsSSc are positive for ACA autoantibodies. SPIN was founded by McGill University researcher Dr. Brett Thombs as a partnership of researchers, scleroderma clinicians, patient organizations, and patients, who work together to develop and test online programs to help people cope with important problems related to scleroderma. SPIN was launched in 2011 with seed funding from the Scleroderma Society of Ontario and Scleroderma Canada.
SPIN maintains a large cohort of over 1,800 scleroderma patients, recruited by scleroderma clinicians from around the world. These patients complete regular online questionnaires to help researchers understand their challenges and support needs. SPIN’s mission is to work with people with scleroderma to identify their needs and prioritize research in areas most important to them and to develop, test, and disseminate accessible patient programs that improve quality of life and empower people with scleroderma and their loved ones. See their letter of thanks in this link. “Mama! Come quick!” my daughter called through sobbing tears. She’d been unwell for a few days and had developed a high fever that morning. I was already on high alert, and her cries for me were like sirens.
As I was crouched down beside her as she lay on the couch, she turned her phone so I could read the alarming message that warned her to seek immediate emergency care. Dr. Google had struck again! With her belly pain and fever, I’d already made a doctor’s appointment for that day. She’d asked why, and I’d told her it was always a good idea to get checked out if you don’t get better after a few days. I hadn’t mentioned that I was worried she had a bladder infection. Just like her mama, she’d wanted to be armed with information. With her phone glued to her teenage hands, she’d turned to the internet for answers. And thanks to Google, she was now terrified her body was close to sepsis and believed a site’s warnings that death was a possibility if she didn’t get to the ER right away. One Google search is all it ever takes to send a sane mind into a death spiral. Autoantibodies called ATAs appear to speed disease progression, study finds by Margarida Maia, PhD from Scleroderma News About half of the people with suspected very early systemic sclerosis (SSc) may go on to develop definite disease, and those who test positive for anti-topoisomerase antibodies (ATAs) are more likely to experience faster disease progression. That’s according to a study from the Netherlands where the proportion of people who tested positive for ATA, autoantibodies associated with SSc, was very low among those with suspected very early disease. “The low ATA prevalence among patients with suspected very early SSc suggests swift disease progression in ATA-positive SSc and consequently a different approach to identify this subgroup before development of irreversible organ damage,” the researchers wrote. The study, “Progression from suspected to definite systemic sclerosis and the role of anti-topoisomerase I antibodies,” was published in the journal RMD Open. Diagnostic workup includes extensive testing SSc, also known as scleroderma, is characterized by self-reactive autoantibodies in the immune system attacking healthy tissues and causing them to become scarred. Scar tissue can build up in the skin and in internal organs such as the heart, kidneys, lungs, and digestive tract. As part of the workup toward diagnosis, patients usually undergo examination for medical history, physical assessments, and various other tests, including blood tests to check for the presence of autoantibodies. Because not all patients meet the criteria for diagnosis, doctors came up with criteria for suspected very early SSc. The criteria consist of Raynaud’s phenomenon (fingers and toes that feel numb, prickly, and frigid in response to cold temperatures or stress), the presence of SSc-specific autoantibodies, puffy fingers, and changes in the small blood vessels under the fingernails. “Specific autoantibodies are important risk factors for progression from suspected very early SSc to definite SSc,” the researchers wrote. In earlier work, however, they found that only a small proportion of patients with suspected very early SSc tested positive for ATA. To know more about ATA and how these autoantibodies contribute to disease progression to definite SSc, the researchers carried out a review of the literature. Percentage of hypochromic red blood cells higher than 2%, DLCO of 65% or lower seen as factors by Marisa Wexler, MS Analyzing the color of red blood cells could help predict survival outcomes among people with systemic sclerosis (SSc), a study shows.
The study, “Hypochromic red cells as a prognostic indicator of survival among patients with systemic sclerosis screened for pulmonary hypertension,” was published in Arthritis Research & Therapy. Red blood cells carry oxygen through the bloodstream out to all the tissues. To ferry it, they use an iron-containing protein called hemoglobin. When iron-rich hemoglobin binds to oxygen, it turns red, which is how blood gets its distinctive “blood red” color. When iron levels are too low, red blood cells can become hypochromic, meaning they’re not as bright. Chronic inflammatory conditions like SSc may cause fluctuations in iron levels, but the clinical consequences are incompletely understood. In this study, researchers led by scientists in Germany assesed whether the percentage of hypochromic red blood cells — referred to as % HRC — was associated with differences in survival among people with SSc. The analysis included data for 171 patients who underwent screening for pulmonary hypertension (abnormally high pressure in the lung’s blood vessels). Among them, 81.3% were female, the mean age was 60, and nearly two-thirds had elevated pulmonary blood pressure. The patients were followed for a median of more than two years, during which time 18 died, mainly from complications related to lung disease. Probiotic therapy may need to be more personalized for autoimmune disorders by Marisa Wexler, MS People with systemic sclerosis (SSc) who have more severe digestive problems tend to have less diversity in the makeup of bacteria living in their gut, a new study reports.
Certain bacterial species, including Lactobacillus — present in commercially available probiotics — are more abundant in SSc patients with more digestive complaints, results show, which suggests that probiotic therapy may need to be more personalized for people with autoimmune disorders like SSc. The study, “Gastrointestinal tract involvement in systemic sclerosis: The roles of diet and the microbiome,” was published in the journal Seminars in Arthritis and Rheumatism. Other risk factors include pneumonia, pulmonary hypertension by Patricia Inácio, PhD from Scleroderma News Systemic sclerosis (SSc) patients with interstitial lung disease (ILD), where the lungs become scarred, are more than three times more likely to be hospitalized due to respiratory failure, according to real-world data collected in the U.S.
Other strong risk factors for respiratory failure among hospitalized SSc patients included pneumonia and pulmonary hypertension, or high blood pressure in the vessels that supply the lungs. “Outpatient optimization and inpatient recognition of these risk factors can lead to improved hospitalization outcomes for SSc patients,” the researchers wrote. The study, “Risk Factors for Respiratory Failure in Patients Hospitalized With Systemic Sclerosis: An Analysis of the National Inpatient Sample,” was published in the journal Cureus. |
AuthorScleroderma Queensland Support Group Archives
May 2024
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