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Are you newly diagnosed? Have someone close to you recently diagnosed and "don't understand" what Scleroderma is? This article will help you. Scleroderma is a chronic disease that affects the skin and connective tissue, the tissue that supports and holds organs together, and is also found in the joints.
The hallmark of scleroderma is thick and hardened skin caused by excessive production of collagen, a protein that is the main component of scar tissue. This scar tissue can accumulate in and damage organs, including the heart and blood vessels, and the lungs, stomach, and kidneys. Females are about four times more likely to develop scleroderma than males. Disease onset typically occurs between ages 35 and 50, though people of all ages can be affected. Causes Scleroderma is an autoimmune disease, meaning it is caused by an overactive immune system that mistakenly attacks the body’s own tissues. The resulting excessive inflammation damages the skin and affected organs. It is a complex condition, and the underlying disease process is not well understood. It is thought that the disease results from a combination of genetic and environmental factors. Scleroderma is not contagious, infectious, or cancerous. Symptoms Symptoms of scleroderma vary among patients, and can range from very mild to life-threatening depending on which parts of the body are affected and the extent of organ involvement. A mild case can become serious if not treated properly. Characteristic symptoms, besides patches of thick and hardened skin, include contractures when skin stiffens over joints, skin ulcers (sores), calcinosis — when lumps of calcium form under the skin — and Raynaud’s phenomenon, in which the fingers and toes feel numb, prickly, or frigid in response to cold temperatures or emotional distress. Manifestations affecting internal organs include damage to muscle and bone tissue, shortness of breath, an abnormal heart rhythm, a burning sensation in the chest (heartburn), difficulty swallowing, blurred vision. A scleroderma renal crisis is possible in some systemic scleroderma (mentioned below) patients. This renal, or kidney, crisis is a serious disorder characterized by a sudden onset of high blood pressure, progressive kidney failure, hypertensive encephalopathy (brain dysfunction or damage due to high blood pressure), and/or microangiopathic hemolytic anemia, or anemia due to the red blood cells being sheared (ripped apart) as they pass through small blood vessels. Study: Inhibitor treatments could help mitigate fibrosis in multiple disorders by Marisa Wexler, MS | November 18, 2025 The molecular signaling pathway EGFR-STAT1 is key for driving fibrosis (scarring) in scleroderma, according to a new study.
The findings suggest that treatments designed to inhibit this pathway could be used to help mitigate fibrosis in scleroderma and other disorders that are characterized by excessive scarring. The study, “EGFR-STAT1 pathway drives fibrosis initiation in fibroinflammatory skin diseases,” was published in Nature Communications. Inhibiting EGFR-STAT1 pathway could help control fibrosis in scleroderma Scleroderma is characterized by inflammation and scarring, which can affect the skin and various other organs throughout the body. Inflammation and scarring are closely linked, but they don’t always occur simultaneously. Some disorders, such as scleroderma, are marked by both inflammation and fibrosis, while others are characterized by inflammation but not fibrosis. In this study, scientists sought to better understand why fibrosis is a feature of some inflammatory skin diseases but not others. The scientists conducted in-depth analyses of cellular activity using samples from people with several different diseases, including disorders that cause scarring, such as scleroderma and lupus, as well as non-fibrotic diseases like atopic dermatitis and psoriasis. “Treatments for fibrosis are an enormously unmet need,” Richard Flavell, PhD, co-senior author of the study at Yale University School of Medicine, said in a university news story. “Better understanding these conditions will likely yield new medicines to help patients.” The researchers found that a signaling protein called STAT1 was substantially more active in patients with fibrotic diseases. Further tests showed that EGFR (epidermal growth factor receptor), a signaling protein that’s previously been implicated in driving fibrosis, is able to activate STAT1, leading to changes in cellular activity that ultimately promote the formation of scar tissue. Study highlights physical, emotional, and social toll of hand impairment by Steve Bryson, PhD | November 4, 2025 Limited hand function makes everyday tasks, work, and hobbies difficult for adults with systemic sclerosis (SSc), according to an interview-based study. The loss of hand mobility and strength also affects emotional health and social life.
Patients said tasks requiring fine motor skills — such as typing or writing — are especially challenging. “Addressing this unmet need requires greater clinical awareness and more personalised and symptom-specific management strategies,” the researchers wrote. The study, “Impact of hand function impairment on daily life of patients with systemic sclerosis: a qualitative study,” was published in Rheumatology. Bristol Myers Squibb tests therapy in autoimmune diseases by Andrea Lobo, PhD | October 28, 2025 A CAR T-cell therapy being developed by Bristol Myers Squibb eased skin thickness and improved lung function in people with systemic sclerosis (SSc), early trial data showed.
These initial results for the treatment, BMS-986353, come from the Phase 1 Breakfree-1 clinical trial (NCT05869955), which is testing the therapy’s tolerability, preliminary efficacy, and pharmacological profile in several autoimmune diseases. Breakfree-1 is currently recruiting at several sites in the U.S. The results are being reported at the American College of Rheumatology (ACR) Convergence 2025, being held Oct. 24-29 in Chicago, in a presentation titled “Efficacy and Safety of BMS-986353, a CD19-Directed Chimeric Antigen Receptor T Cell Therapy Manufactured Using a Next-Generation Process: Updated Data From a Phase 1 Trial in Patients With Systemic Sclerosis.” “While early, these data demonstrate our focus and steady advancement toward introducing the potential of treatment-free remission, which just a few years ago was not thought to be possible for patients with severe autoimmune disorders,” Lynelle B. Hoch, president of the cell therapy organization at Bristol Myers Squibb, said in a company press release. SSc is caused by an overactive immune system that results in inflammation and accumulation of scar tissue in the skin and connective tissues, which support and hold organs together. Immune B-cells produce antibodies to help the body fight off diseases, but may also produce self-reactive antibodies that drive autoimmune disease. |
AuthorScleroderma Queensland Support Group Archives
November 2025
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