Scleroderma (systemic sclerosis) is a chronic autoimmune disease caused, in part, by the body’s overproduction of collagen. It is classified as a rheumatic disease, meaning it affects the body’s connective tissues and structures and even underlying organs. Scleroderma is also considered a progressive condition, meaning it gets worse over time.

Your body’s connective tissues are composed of a variety of proteins, cells and other materials. Collagen is the main structural protein in a number of these connective tissues, helping to provide strength and stability to joints, tendons, and ligaments. Too much collagen, however, can cause the skin to stretch and harden. It can also cause issues with the structures beneath the skin.

Here’s everything you need to know about scleroderma, a painful and often debilitating rheumatic disease that is characterized by skin thickening:

Different Types of Scleroderma

There are two categories of scleroderma: systemic and localized. Within the systemic category, scleroderma can be classified as diffuse or limited. Within the localized category, scleroderma can be classified as morphea or linear.

Localized scleroderma typically affects only the skin. Morphea and linear refer to the patterns in which scleroderma patches appear on the skin. Systemic scleroderma (systemic sclerosis) affects both the body’s skin and connective tissues and underlying organs.

Sclerosis refers to the “hardening” of multiple organ systems throughout the body. Diffuse systemic scleroderma is characterized by more widespread skin hardening and a greater likelihood of developing fibrosis of the internal organs.

CREST syndrome, also known as limited scleroderma, typically affects the skin and organs more slowly than diffuse scleroderma.

What Causes Scleroderma?

There are a number of factors that contribute to the development of scleroderma. The most common factors include genetics and environmental triggers like exposure to certain viruses or chemicals. Scleroderma is more likely to occur in women than men, and typically affects people between the ages of 30 and 50.
Many people with the disease also exhibit symptoms of other autoimmune disorders, like lupus or rheumatoid arthritis.

What do scientists and researchers know about the mechanics of the disease?

Unfortunately, scleroderma’s exact etiology (causation) is unknown. It is one of the most complex autoimmune conditions, displaying both innate and adaptive immune system abnormalities.

Scleroderma Symptoms

The earliest symptom of scleroderma is change in the skin. Hardening, tightening and thickening of the skin are the hallmarks of the disease.

Key overall symptoms of scleroderma include:

  • Thick, hardened skin – In scleroderma, thickened skin can appear on any part of the body. The shape and size of these hardened skin patches vary widely from person to person. This skin is often so taut that it appears shiny, and the area of the body covered by the skin may be difficult to move.
  • Hard lumps on the skin called calcinosis
  • Small, red bumps on the skin (telangiectasia) – Widened blood vessels occur in approximately 75% of people with scleroderma .
  • Narrowed blood vessels (especially in hands and feet) – Raynaud’s Phenomenon is a common side effect of scleroderma . The disease can cause the blood vessels in the hands and fingers to spasm in response to changes in temperature or emotional triggers. Compromised blood vessels reduce blood flow to the hands, causing numbness, discoloration and discomfort.
  • Digital ulcers – Scleroderma often compromises blood vessels and blood supply, especially in the hands, feet, fingers and toes. As a result, ulcers and open wounds can form on these parts of the body. Around 40% of people with scleroderma develop open sores on their fingers.
  • Swollen joints
  • Joint pain and tenderness (inflammation)
  • Digestion issues – Nausea, heartburn, appetite changes, and constipation
  • Lung fibrosis – Thickening or scarring of lung tissues occurs in a large percentage of people with scleroderma. Over time, lung fibrosis can make it more difficult for oxygen to reach the bloodstream. This can lead to pulmonary hypertension.
  • Myocardial fibrosis (scarring around the heart)
  • Kidney issues – Kidney fibrosis is fairly common in scleroderma and can compromise the kidneys’ ability to function. A small percentage of people with scleroderma develop a serious medical condition called renal crisis.

If you exhibit any of these symptoms, it’s important to visit your doctor immediately.

How is Scleroderma Diagnosed?

Scleroderma is notoriously difficult to diagnose. The condition is often mistaken for similar diseases like rheumatoid arthritis or lupus. However, with a combination of examination and testing, diagnosis and successful symptom control is possible. In addition to collecting your full medical history, your doctor will perform a medical examination. He or she may also order blood tests to check for levels of autoimmune antibodies. Finally, you may need to donate a skin sample to be tested for indicators of scleroderma.

Additional tests that may be required:

  • Pulmonary function test
  • EKG
  • Echocardiogram
  • Gastrointestinal motility test
  • CT scan

How is Scleroderma Treated?

There is no cure for scleroderma, but with proper treatment, symptoms can be managed.

Pain relief is an important aspect of scleroderma management. NSAIDs and anti-inflammatory medications are often prescribed for management of SS-related pain.

Topical creams and ointments may be recommended to control skin itching and discomfort.

Physical therapy may be recommended to improve joint and muscle-related symptoms, while other medications can help control blood pressure and gastrointestinal issues.

The most important step you can take in controlling the symptoms of scleroderma is to find a reliable rheumatologist as soon as possible.

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