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.
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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.”
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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.
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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.
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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.

Mouse study shows the tiny natural particles reduced inflammation by Margarida Maia, PhD | October 21, 2025

Milk-derived extracellular vesicles — very small, natural particles from cow’s milk — were shown to safely reduce inflammation and scarring in a mouse model of systemic sclerosis (SSc).

Because these vesicles carry molecules that can protect and help tissue repair itself, they “have attracted much attention due to their high yield, easy availability, and high content, which endows them with great potential for clinical applications,” the researchers wrote.

The study, “Blockade of TGF-[beta]1/Smad3 signaling pathway by bovine milk-derived extracellular vesicles ameliorates skin fibrosis in systemic sclerosis mice,” was published in Biochemical and Biophysical Research Communications by scientists in China.
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Scleroderma occurs when the immune system prompts the body to make an excess of collagen that thickens and hardens the skin and internal organs. In scleroderma, the connective tissue becomes stiffened and swollen.

Analysis: Protein markers and disease duration strongly predict MMF response
by Margarida Maia, PhD | October 14, 2025

In people with diffuse cutaneous systemic sclerosis (dcSSc), certain skin features, including levels of specific molecules and cells, depend on disease duration, a study finds.

These features, combined with disease duration, allowed researchers to predict patients’ response to mycophenolate mofetil (MMF), an immunosuppressive therapy commonly used for dcSSc.

These findings suggest “skin biopsies may be useful for refining prognosis and guiding patient management decisions,” the researchers wrote.

​The study, “Skin biopsies enhance prediction of clinical trajectory in diffuse cutaneous systemic sclerosis,” was published in Arthritis & Rheumatology.

More than a third of patients saw progressive lung disease, study found by Margarida Maia, PhD | October 7, 2025

​More than a third of adults with systemic sclerosis (SSc) who develop interstitial lung disease (ILD) see their condition progress, and those who reported increasing shortness of breath in the past month had a higher risk of death — even if their breathing tests have not declined — an Australian study finds.

“This study has highlighted the prognostic importance of patient-reported symptoms in the evaluation of SSc-ILD,” the researchers wrote in “The prognostic importance of worsening dyspnoea in systemic sclerosis related interstitial lung disease,” which was published in Seminars in Arthritis and Rheumatism.
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In SSc, the immune system mistakenly attacks its own tissues, leading to the formation of fibrotic (scar) tissue. When this occurs in the lungs, it can appear as ILD, where the lung tissue becomes inflamed and stiff. This makes it harder for air to move in and out of the lungs, making it difficult to breathe.

Researchers say they could serve as biomarkers, preventive strategy targets
by Margarida Maia, PhD | September 30, 2025

​Researchers in China have identified five genes — COL1A2, COL3A1, COL15A1, THY-1, and CCL19 — that may play a key role in lung complications associated with systemic sclerosis (SSc), according to a study.

Using bioinformatics analyses of patient tissue samples, the team found that these genes were consistently overactive and linked to processes such as cell migration, biological adhesion, and collagen biosynthesis, which contribute to tissue scarring and increased blood pressure in the lungs.

Researchers suggest that these genes could serve as potential markers for early diagnosis and as targets for preventive or therapeutic strategies against SSc-associated lung disease.
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The study, “Identification and validation of key genes commonly expressed and upregulated in systemic sclerosis-associated lung diseases through integrated analysis,” was published in Biochemical and Biophysical Research Communications.

Living with a chronic illness is like fighting a war by Tomisa Starr | September 24, 2025

​When I was diagnosed with scleroderma, I cried. I wondered, “What is going to become of me?” I didn’t feel brave. I felt helpless and afraid.

My scleroderma symptoms didn’t come all at once. Shortness of breath and fatigue — symptoms of interstitial lung disease (ILD) — came on slowly. These can delay an ILD diagnosis because they are vague and can have many causes. For example, I have pulmonary arterial hypertension as well as scleroderma, and difficulty breathing and fatigue can also be caused by a scleroderma renal crisis. In my case, however, the symptoms are caused by scleroderma’s direct effect on my lungs. 
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I also have gastrointestinal symptoms, such as heartburn and bloating, that are very bad and my reflux is unbelievably painful. Whenever I have reflux, my throat hurts so badly that it feels like I’ve been scalded. Drinking water helps thin out the mucus and acid, but it still hurts. I take medications to control my stomach acid.

Changes in protein signatures a potential therapeutic target by Steve Bryson, PhD | September 16, 2025

Increasing levels of self-reactive antibodies in the bloodstream after the start of treatment are associated with the progression of systemic sclerosis (SSc), according to recent research.

“We hypothesize that rising antibody [levels] during treatment may reflect ongoing immune activation, insufficient therapeutic response, or impending flare, whereas decreasing [levels] may signal immune modulation and treatment efficacy,” researchers wrote.

Moreover, changes in protein signatures related to the metabolism of fat-like lipids and oxidative stress, a type of cellular damage, may drive SSc progression, “highlighting novel biomarkers and therapeutic targets for personalized SSc management,” they added.
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The biomarker study, “Longitudinal Autoantibody and Proteomic Profiles Uncover Biomarkers and Mechanisms of Disease Progression in Systemic Sclerosis,” was published in Arthritis & Rheumatology.