What is Scleroderma?

Scleroderma (also known as systemic sclerosis) is an autoimmune condition that causes the hardening of the body's connective tissue (e.g. skin, lungs, kidney, etc.). According to Scleroderma Australia, the thickening of the connective tissue can become severe and even affect the organs. More than 6000 Australians live with scleroderma. The condition peaks between the ages of 40-60 and are three times more common in women. Scleroderma is also not genetic and is rare in more than one family member [1]. Awareness about scleroderma is essential to help the community recognise and support the condition. According to Scleroderma Australia, the 29th of June is World Scleroderma Day. This is a day that helps bring awareness and raise funds for research, engagement and the community.

Types of Scleroderma

There are two types of scleroderma called localised and systemic scleroderma. Localised scleroderma usually causes hardening and thickening of the skin rather than the organs. On the other hand, systemic scleroderma affects layers of connective tissue, including the skin, blood vessels and major organs (e.g. intestines, heart, lungs, etc.) [2].

Scleroderma Symptoms

Symptoms of scleroderma vary between individuals. For instance, those with localised scleroderma will usually have changes to their skin and the nearby tissue. Patches of discolouration commonly occur in areas such as hands, fingers and face. Whereas those with systemic scleroderma have more widespread symptoms, including [2]:

Raynaud's phenomenon (a reaction to changes in temperature that leads to discolouration of particular body parts, including fingers and nose.
Respiratory and breathing problems, such as interstitial lung disease
Stomach issues, such as gastrointestinal oesophageal reflux (GORD)
Heart problems
Kidney issues
Tendon and joint pain

What Causes Scleroderma?

Scleroderma is an autoimmune disease caused by the immune system attacking the body's connective tissue [3]. At the moment, researchers are still investigating what causes this to happen. However, it is understood that people with this condition experience excessive growth of collagen and body tissue [4]. This leads to thickening, tightening and scarring along the skin and/or organs. However, certain risk factors may increase the chance of developing scleroderma, including specific genetic changes and exposure to certain compounds (e.g. silica) [2].

Tests and Diagnosis

If you suspect that you may have scleroderma, please consult a GP for a diagnosis and/or further testing. A medical screening involving questioning, a review of your medical history and/or a physical examination may be required. There is no single test that will be able to diagnose scleroderma. Your GP may also refer you to a rheumatologist, dermatologist and/or other specialists for further assessment. As a result, several assessments may be performed or referred by your doctor, including:

Blood Testing

Your doctor or rheumatologist may order blood tests to look for antinuclear antibodies (ANA) [5]. A positive ANA screening may suggest the presence of an autoimmune condition. However, further testing may be required for a diagnosis.

Nailford Capillary Test

Using a device called a capillaroscope, your GP and/or specialist will look under the finger for any changes to the blood vessels underneath [5]. Abnormal changes to these blood vessels may be a sign of scleroderma.

Scleroderma Skin Pinch Test

A doctor will examine your skin by lightly pinching several body areas, such as the face, arms, thigh and feet. This test will determine the thickness of the skin around the body. These measurements will be scored to help diagnose and monitor the progression of the condition [5].

Pulmonary Function Test

Tests, such as spirometry and CT scans, can be performed to monitor the health of the lungs [5]. Those with scleroderma can develop serious lung conditions, such as interstitial lung disease or pulmonary arterial hypertension. These tests can measure the health of the lungs and detect these complications.

Heart Tests

An echocardiogram (ultrasound imaging of the heart) or MRI can be performed over the chest to see how the heart is functioning. These tests will help monitor any abnormal changes to the heart, which are rare but severe complications of scleroderma [5].

Upper Gastrointestinal Tests

The upper gastrointestinal region is made up of areas such as the mouth, oesophagus, stomach and small intestines. Complications to the upper gastrointestinal tract, such as reflux, ulcers and bleeding, can occur in those diagnosed with this condition [5]. Tests, such as endoscopies and colonoscopies, can monitor the health of these areas.

Scleroderma Treatments

Currently, there is no cure for scleroderma. Your doctor may suggest several treatment options depending on your circumstances and symptoms.

Medications

Your doctor may prescribe medications such as immunosuppressant drugs, pain relief and/or corticosteroids to control your condition. Topical medications (which are applied to the skin) can also help with plaques and growths on the skin. Always use the medication as directed.

Skin Lotions and Moisturisers

Scleroderma can cause dry and itchy skin, which can be highly uncomfortable. Skin lotions and moisturisers can be applied to help relieve symptoms.

Other Treatments

Managing diet and nutrition to help with gut health
Physiotherapy to help strengthen and stretch the body
Seek psychology to assist with any changes in emotion and mental states
Surgery, if appropriate and when other treatments do not help
Laser therapy for skin treatment

Recovery

Those with localised scleroderma usually recover within 3-5 years. The hardened and thickened skin will begin to clear on its own [6]. Systemic scleroderma is considered a chronic condition that cannot be cured. Depending on the severity and the nature of the condition, the symptoms can be treated. Speak to your doctor about treatment options that can help prolong a high quality of life and to help reduce the progression of the condition.

Complications

Due to the hardening of the skin, those with scleroderma may experience functional difficulties, such as stiffness and opening the mouth. Additionally, skin changes can lead to self-consciousness and psychological issues [2]. Additionally, those with systemic scleroderma may experience more significant organ complications. Fibrosis (scarring) of the connective tissue of the internal organs and structures can lead to conditions such as [2]:

Inflammatory arthritis
Interstitial lung disease
Pulmonary arterial hypertension
Arrhythmias
Raynaud's phenomenon
Kidney problems
Gut issues

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