Aug 17, 2023
Scleroderma, also known as systemic sclerosis, is a term used to describe a group of rare diseases that cause the skin to become stiff and tight. Furthermore, the blood vessels, internal organs, and digestive system may all be impacted.
It only describes how much skin is affected when scleroderma is called "limited" or "diffuse," respectively. Both types could be vascular or involving any other organs. Localised scleroderma, also known as morphea, has effects on the skin alone.
Therapy can lessen symptoms, stop the disease's course, and improve quality of life, but there is no known cure for scleroderma.
Overproduction and buildup of collagen in bodily tissues cause scleroderma. Your skin and other connective tissues in your body are composed of collagen, a fibrous kind of protein.
Although the actual etiology of this process is unknown to doctors, the immune system of the body seems to be involved. Scleroderma is probably brought on by a mix of immune system issues, genetic predispositions, and environmental triggers.
Along with the thickening of the skin, scleroderma patients may also experience the following symptoms:
Along with these symptoms, people with scleroderma also have Raynaud's phenomenon and Sjögren's syndrome. Scleroderma patients that experience Raynaud's phenomenon account for 85% to 95% of cases. On the other hand, primary Raynaud's syndrome is common and frequently appears on its own without any underlying connective tissue disorder.
Scleroderma will only appear in 10% of Raynaud's phenomenon patients.
Dry mouth and eyes are signs of Sjögren's syndrome. This dryness is brought on by the absence of tears and saliva as a result of immune system damage and the destruction of the glands that produce moisture in the body.
Everyone can get scleroderma, but women are much more likely to get it than men are. The likelihood of getting scleroderma appears to depend on a number of interrelated factors, including:
Scleroderma can be challenging to diagnose since it can manifest in so many various ways and impact so many different body parts.
Your doctor may recommend blood testing after performing a complete physical examination to look for high levels of certain antibodies produced by the immune system.
In order to assess whether your digestive system, heart, lungs, or kidneys are harmed, your doctor may also recommend additional blood tests, imaging scans, or organ-function testing.
The excessive collagen production that is a hallmark of scleroderma cannot be cured or stopped by any treatment. The symptoms can be managed and consequences can be avoided, though, with the aid of several treatments.
Scleroderma can affect so many various body areas that the type of treatment will fluctuate based on the symptoms. A few examples are medications that:
You can increase your strength and mobility with the aid of physical or occupational therapists, maintaining your freedom while doing so. Hand therapy may be able to stop contractures in the hands.
Stem cell transplants may be an option for patients whose severe symptoms have not improved with more traditional therapy. Organ transplants might be a possibility if the kidneys or lungs have suffered substantial damage.
Mild to severe scleroderma consequences can affect the following:
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