Scleroderma, or systemic sclerosis, encompasses a cluster of uncommon diseases characterised by the tightening and hardening of the skin. It can give rise to complications in blood vessels, internal organs, and the digestive tract.

In addition to its primary impact on the skin, scleroderma can also affect various other parts of the body, including the muscles, digestive system, lungs, kidneys, blood vessels, and heart. 

Although a cure for scleroderma has yet to be discovered, there are treatments available to alleviate symptoms, decelerate progression, and enhance the quality of life.

What is Scleroderma?

Scleroderma,  also known as systemic sclerosis,  is a chronic autoimmune disease that causes abnormal growth of connective tissue in the body called collagen. Collagen is a protein that gives the skin, bones, and other tissues their strength and flexibility. This excess collagen production leads to the hardening and tightening of the skin and can affect various organs and systems within the body. It is believed to result from an overactive immune system attacking healthy tissues.

Scleroderma is a rare disease and can affect people of any age, but it is most common in adults between the ages of 30 and 50. 

Types of Scleroderma:

Scleroderma exists in various forms, each with its own unique impact on different areas of the body, either locally or systemically.

Localised Scleroderma: It is the more prevalent form, primarily affecting the skin, usually in limited areas. In some cases, this type of scleroderma may resolve without treatment, but it can also progress into a systemic condition.

Systemic Scleroderma: On the other hand, diffuse scleroderma, also referred to as systemic scleroderma, impacts multiple areas of the body. This form of the condition is considered more severe. Without appropriate intervention, it can lead to significant complications and pose life-threatening risks.

CREST syndrome: In CREST syndrome or limited scleroderma skin thickening is restricted to specific areas such as the fingers, hands, and forearms, and digestive disturbances primarily affect the esophagus. CREST syndrome is an acronym encompassing the symptoms associated with this more restricted manifestation of the disease, which includes Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia.

Causes of Scleroderma

The exact cause of scleroderma remains uncertain to doctors. The symptoms arise from the excessive production and buildup of collagen, yet the underlying reasons behind the body's overproduction of collagen are not well understood. Nevertheless, there are several recognized risk factors that can increase an individual's susceptibility to developing this condition. The risk factors for scleroderma include the following:

Genetics: Genetics plays a significant role in the development of scleroderma. Individuals with specific gene variations have a higher likelihood of developing the condition. This genetic predisposition may elucidate why scleroderma can be observed in families and certain ethnic groups.

Immune System Issues: Scleroderma is classified as an autoimmune disease, wherein the body's immune system mistakenly attacks its own tissues. Interestingly, around 20% of individuals diagnosed with scleroderma also experience symptoms associated with other autoimmune conditions like rheumatoid arthritis, Sjogren's syndrome, and/or lupus.

Environmental Triggers: Although it is considered the least common factor, it is important to acknowledge environmental triggers in relation to scleroderma. Certain studies propose that symptoms of scleroderma may be triggered by exposure to specific viruses, medications, or drugs. Furthermore, repeated contact with certain harmful substances and chemicals can elevate the risk of developing this condition.

Symptoms of Scleroderma

The symptoms of scleroderma can vary in intensity and presentation, contingent upon the specific type and severity of the disease. In the case of systemic scleroderma, some prevalent symptoms often observed include:

• Skin hardening and tightening

• Raynaud's phenomenon (a condition that causes the fingers and toes to turn white, blue, or red when exposed to cold)

• Swollen hands and feet

• Joint pain and stiffness

• Chest pain

• Shortness of breath

• Trouble swallowing

• Digestive problems

• Kidney problems

• High blood pressure

The symptoms of localised scleroderma are usually limited to the skin. Some common symptoms of localised scleroderma include:

• Hard, smooth patches of skin

• Wrinkles and scarring

• Mottled skin colour

• Hair loss

Treatment Options:

While there is no cure for scleroderma, treatment focuses on managing symptoms, preventing complications, and improving quality of life. The treatment approach may vary depending on the specific symptoms and organs affected. Some common treatment options include:

• Pain relievers

• Anti-inflammatory drugs

• Immunosuppressants

• Vasodilators (drugs that relax the blood vessels)

• Physical therapy

• Occupational therapy

• Speech therapy

Conclusion:

Scleroderma is a chronic autoimmune disease that can have a significant impact on a person's physical and emotional well-being. While a cure for scleroderma does not exist, timely detection and suitable treatment can effectively control symptoms, minimise complications, and enhance overall quality of life. It is crucial for individuals living with scleroderma to understand that it is a chronic condition rather than an imminent threat to life. By implementing appropriate treatment strategies and adopting proper management techniques, individuals with scleroderma can lead extended and satisfying lives.