Scleroderma: Causes, Symptoms, Risk Factors, Diagnosis, Treatment and Complications

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.

Cause Of 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.

Symptoms Of Scleroderma

Along with the thickening of the skin, scleroderma patients may also experience the following symptoms:

• The hands and feet swelling
• Telangiectasias, red skin lesions
• Calcinosis, or excessive cutaneous calcium deposition
• Stiff joint contractures
• Face with tight, mask-like skin
• Bruises on the toes and fingers
• Stiffness and discomfort in the joints
• Ongoing cough
• Breathing difficulty
• Indigestion (acid reflux)
• Having trouble swallowing
• Gastrointestinal and digestive issues
• Constipation
• Weight loss
• Fatigue
• Hair fall

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.

Risk Factors Of Scleroderma

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:

• Genetics. There seems to be a link between scleroderma development and specific gene variants. This may help to explain why some scleroderma cases appear to run in families and why some ethnic groups are more likely than others to develop particular types of scleroderma.
• Environmental Causes. According to research, exposure to specific viruses, medicines, or treatments may in some cases cause the symptoms of scleroderma. Scleroderma risk may also rise with repeated exposure to specific dangerous agents or chemicals, such as those present at work. Most folks do not have a specific environmental trigger.
• Immune system problems. It is believed that scleroderma is an autoimmune disorder. Therefore, it follows that the immune system of the body targeting the connective tissues is a contributing factor. Patients with scleroderma could also have symptoms of lupus, rheumatoid arthritis, or Sjogren's syndrome.

Diagnosis Of Scleroderma

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.

Treatment Of Scleroderma

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.

Medications

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:

• Stretch the blood vessels. Blood vessel-dilating blood pressure medicines may be used to treat Raynaud's syndrome.
• Minimize the immunological system. Immune-suppressing drugs, such as those provided after organ transplants, may decrease the progression of some scleroderma symptoms, such as skin thickening or worsening lung damage.
• Stomach Discomfort is lessened. By using medication to reduce stomach acid, heartburn can be eased.
• Eliminate infections. Fingertip ulcers brought on by Raynaud's illness may not become infected with proper cleaning and cold weather protection. Immunizations against the flu and pneumonia on a regular basis can help protect lungs that have been harmed by scleroderma.
• Reduce discomfort. Your doctor may recommend prescription painkillers if over-the-counter analgesics are ineffective.

Therapies

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.

Various Surgical and Other Processes

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.

Complications Of Scleroderma

Mild to severe scleroderma consequences can affect the following:

• Fingertips. The restricted blood flow can become so bad in people with Raynaud's syndrome and systemic sclerosis that it permanently damages the tissue in their fingertips, resulting in skin ulcers or pits. Sometimes the tissue on the fingertips will lose its integrity.
• Lungs. Your capacity to breathe and tolerance for exercise may be affected by lung tissue scarring. It's also possible that the arteries leading to your lungs have high blood pressure.
• Kidneys. Scleroderma renal crisis, a dangerous kidney condition, causes a fast rise in blood pressure and quick kidney failure. To maintain renal function, this problem must be treated quickly.
• Heart. Your risk of irregular heartbeats and congestive heart failure increases if your heart tissue becomes scarred.
• Additionally, the membranous sac that surrounds your heart may become inflamed as a result of scleroderma.
• Teeth. Your mouth may become smaller and narrower due to severe facial skin tightness, making it difficult to wash your teeth or even have them professionally cleaned. The likelihood of dental decay is increased in those with scleroderma since they frequently don't generate regular amounts of saliva.
• Intestinal system. Heartburn and swallowing issues might be brought on by digestive issues linked to scleroderma. Cramping, bloating, constipation, and diarrhea are other possible side effects. A bacterial overgrowth in the intestines in some patients with scleroderma may make it difficult for them to absorb nutrition.
• Joints. Particularly in older people, the skin over joints can tighten to the point that it prevents joints from moving freely.

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