Systemic sclerosis (also known as scleroderma) causes thickening, hardening and scarring of the skin and organs. Dr Sue-Ann Ng, Consultant from the Department of Rheumatology & Immunology at Singapore General Hospital (SGH), explains.
What is systemic sclerosis?
“In systemic sclerosis, the body’s faulty immune system stimulates the fibroblast cells to produce excess collagen leading to thickening, hardening and scarring of the skin and organs,” shares
Dr Sue-Ann Ng, Consultant from the
Department of Rheumatology & Immunology at
Singapore General Hospital (SGH), a member of the
Systemic sclerosis is an autoimmune disease characterised by abnormalities of the blood vessels, connective tissue and the immune system. It can affect the skin, digestive system, heart, lungs, muscles and joints.
Systemic sclerosis affects an estimated 20 to 200 people per million worldwide, with the
age of onset usually between 30 to 50 years.
Women are 4 times more likely to develop the condition than men.
There are two subtypes of systemic sclerosis:
Limited systemic sclerosis: Skin involvement is less widespread (typically confined to the fingers, hands, lower arms, legs and face). Excess collagen deposits cause narrowing of small blood vessels and decreased blood flow to the extremities, such as the fingers and toes, which often turn white / blue in response to cold temperatures or emotional stress (known as Raynaud's phenomenon). Raynaud’s phenomenon often precedes the skin involvement by many years. Late manifestations may include
pulmonary arterial hypertension (increased blood pressure in the artery that carries blood from the heart to the lungs).
Diffuse systemic sclerosis: Skin involvement is more widespread and with a much faster onset and progression than limited systemic sclerosis. Raynaud’s phenomenon often occurs within one year of skin changes. Diffuse systemic sclerosis leads to a higher risk of hardening of the internal organs such as the lungs, kidneys and heart.
What causes systemic sclerosis is currently unknown. It is not contagious and not hereditary. Whilst there are some susceptibility genes which increase the risk of getting systemic sclerosis, the genes themselves do not cause the disease. An environmental trigger, in a person who is at risk, may result in the disease manifesting.
Systemic sclerosis: Early symptoms
The first sign of systemic sclerosis is usually the development of areas of
thickened or hard skin around the joints of the fingers and toes. This hardened skin tends to lead to skin tightening that may decrease joint mobility. There may be intense skin itching and inflammation.
Patients may also experience symptoms such as:
Joint pain and swelling (similar to arthritis)
Shortness of breath or persistent coughing (if the lungs are affected)
Fatigue with very high blood pressure (if the kidneys are affected)
Chest pains or irregular heart beat (if the heart is affected)
Heartburn, swallowing difficulties, constipation, diarrhoea, weight loss (if the digestive system is affected)
Dry mouth and eyes (if salivary glands and tear ducts are affected)
Dental health may also be compromised due to difficulty in opening the mouth caused by tight facial skin and dry mouth may lead to tooth decay.
Systemic sclerosis: How to diagnose
The most common test for systemic sclerosis is an
immunological blood test that detects antibodies produced by the body that are specific for systemic sclerosis. Occasionally a skin biopsy or examination of the small blood vessels of the nailbed under the microscope is required.
Other tests may include X-rays, CT scan, echocardiogram (heart scan), lung function test, endoscopy (to look at the oesophagus and stomach) and urine tests, depending on whether other internal organs are affected.
Systemic sclerosis: How to treat
There is currently no cure for systemic sclerosis. Specialised and dedicated systemic sclerosis clinics are shown to result in better outcome. Sustained and increasing research is therefore necessary. Treatment is based on alleviating the different symptoms of the condition and limiting the damage to the internal organs through medication.
Pain relief for stiff and swollen joints is usually possible with non-steroidal anti-inflammatory drugs. Regular moisturiser can help areas of dry and irritated skin. Antihistamines may help with intense itching of the skin.
For oesophageal involvement, H2 blockers, proton pump inhibitors or prokinetics may be prescribed.
When a patient is diagnosed with diffuse systemic sclerosis, medication may be prescribed to decrease the activity of the immune system. When internal organs are affected by the condition, other specific treatments may be needed.
Systemic sclerosis: What to do if you have it
Regular moderate exercise is important to improve joint flexibility and cardiovascular health. Consult a doctor or physiotherapist about the type of exercise you can do.
For Raynaud’s phenomenon, it is important to keep warm and to stop smoking. Medication to improve blood may be prescribed, while antibiotics can protect skin ulcers from getting infected. Proper wound care to promote ulcer healing can help to slow down the progression in systemic sclerosis.
For the digestive system, simple measures such as avoiding acidic foods, eating smaller and more frequent meals, elevating the head of the bed and avoiding lying down for at least three hours after a meal can help to decrease symptoms of acid reflux.
Check out other articles on autoimmune diseases (rheumatic diseases):
Autoimmune Diseases (Rheumatic Diseases): Types and Symptoms
Rheumatoid Arthritis: Symptoms and How to Treat
Facts About Lupus: Who's At Risk?
Spondyloarthritis: An Inflammatory Rheumatic Disease
Osteoarthritis: Pain and Inflammation of the Joints
Autoimmune Disorders: Frequently Asked Questions