Systemic Scleroderma: Types, Causes, Symptoms, Diagnosis

Exploring Systemic Scleroderma: Types, Symptoms & Care Strategies

Systemic scleroderma is a rare, long-term condition that affects the skin, blood vessels, and internal organs of an individual. It can be confusing and overwhelming at first, especially if you or a loved one has just been diagnosed. However, understanding the basics can help ease that fear and provide a clearer picture of what actions can be taken.

What is systemic scleroderma?

Systemic scleroderma, also called systemic sclerosis, is a chronic autoimmune disorder in which the immune system mistakenly attacks the body’s healthy tissues. This leads to excessive collagen production, which is a protein essential for skin strength and flexibility.

When collagen is overproduced, it causes the skin to become thickened, tight, and hard. Over time, this buildup can extend beyond the skin to affect vital internal organs such as the lungs, heart, kidneys, and digestive tract, impairing their function.

Unlike localised scleroderma, which affects only the skin, systemic scleroderma involves widespread complications and requires comprehensive medical attention to manage symptoms and limit organ damage.

What Causes Systemic Scleroderma?

The exact cause of systemic scleroderma is not clearly known. However, researchers found that a mix of genetics, environmental triggers, and problems with the immune system plays a crucial role. Here are the details:

• Genetics: Scleroderma is not directly inherited, but people with a family history may have a higher risk of developing the condition. Certain genes can increase the potential of developing scleroderma.
• Environmental Factors: Researchers believe that exposure to certain chemicals or substances can potentially trigger or increase the risk of scleroderma.
• Immune System Changes: The overactivity and inflammation of the immune system can trigger cells to produce excess collagen, leading to the characteristic tissue thickening.
   

It is important to remember that systemic scleroderma is not contagious and cannot be passed from person to person through contact.

Types of Systemic Scleroderma

There are three main types of systemic scleroderma based on the extent of skin involvement and how quickly symptoms appear. They are:

1. Limited Cutaneous Systemic Sclerosis: Skin thickening is confined to the hands, feet, face, and forearms.
2. Diffuse Cutaneous Systemic Sclerosis: Skin thickening affects the trunk and upper arms/thighs in addition to distal areas.
3. Systemic Sclerosis Sine Scleroderma: Patients have characteristic internal organ involvement but no skin thickening.

Symptoms of Systemic Scleroderma

Symptoms can vary widely depending on which parts of the body are affected. They can range from mild to severe and often develop gradually. Common symptoms of systemic scleroderma include:

• Skin changes such as tight, thickened, or shiny skin, especially on the fingers, hands, and face.
• Raynaud's phenomenon is where fingers and toes turn white or blue in response to cold or stress.
• Shortness of breath or a dry cough
   

Other common symptoms include joint pain and digestive issues like heartburn and difficulty swallowing.

What is the diagnosis of systemic scleroderma?

Diagnosis of systemic scleroderma can be tricky, especially in the early stages. Doctors usually begin with a physical exam and a review of symptoms. Some common tests conducted to confirm the condition include:

• Blood tests to check for specific antibodies associated with the disease
• Skin biopsies to examine tissue samples
• Pulmonary function tests
• Imaging scans such as chest X-rays or CT scans to assess the lungs
   

Early diagnosis is key to managing the disease and preventing further complications.

Managing and Treating Systemic Scleroderma

Currently, there is no cure for systemic scleroderma, but there are various treatments available that can help manage the symptoms. This includes:

• Medications are commonly used for specific conditions such as blood pressure, Raynaud’s and kidney problems.
• Immunosuppressants can help calm the immune system.
• Specific skin treatments to soften and hydrate the skin may be recommended.
• Physical therapy is also beneficial for maintaining joint flexibility and improving movement.
   

Additionally, maintaining good skincare routines and using moisturisers can help protect the skin. Lifestyle changes, such as avoiding cold temperatures and eating smaller, more frequent meals, may also help manage symptoms.

Systemic scleroderma is a rare and complex autoimmune disease that affects both the skin and internal organs. While the exact cause of the disease is unclear, it is believed to be influenced by genetic, environmental, and immune system factors. Early diagnosis and appropriate treatment can make a significant difference in managing symptoms and improving quality of life.

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