What is Systemic Sclerosis?

Systemic Sclerosis or Scleroderma is a multisystem autoimmune disease characterized by vasculopathy (abnormal blood vessels), and fibrosis of the skin and the internal organs, commonly the lungs, heart and gut. The condition is divided into 2 main types: “Diffuse” and “Limited”, depending on the extent of skin thickening. In Diffuse Cutaneous Systemic Sclerosis, the skin thickening extends from fingers to above the elbows or from toes to above the knees and body; but in Limited Cutaneous Systemic Sclerosis, the skin thickening extends from the fingers to below the elbows or from the toes to below the knees. Both types may have facial skin thickening. Limited type is more likely to have heart involvement, while diffuse type is more likely to have lung involvement.

What are the symptoms of Systemic Sclerosis?

The most common symptom is Raynaud’s phenomenon: where a patient’s fingers or toes change colour to white, then to blue in cold environment, and to red when in warmer environment. Other symptoms include thickening of the skin, joints stiffness and pain, mouth becoming smaller, reflux, heart burn, difficulty in swallowing, and red rashes over the face, neck or hands. In more chronic or advanced cases, patients may have painful ulcers of the fingers or toes, cough and shortness of breath when walking or at rest, abdominal bloating, recurrent diarrhoea or constipation, and loss of weight.

What are the treatments available for Systemic Sclerosis?

Once the diagnosis of Systemic Sclerosis is made, various tests (including blood and urine tests, lung function tests, Chest X-ray, HR CT thorax, and echocardiograph) need to be done to evaluate the extent of organ involvement. Besides regular follow up with blood and urine tests, patients with systemic sclerosis need yearly screening with pulmonary function test to monitor for lung involvement, and echocardiography for heart involvement.
Treatment of systemic sclerosis depends on which organs are involved and the severity of the involvement.

A variety of immunosuppressants such as traditional immunosuppressants, anti-CD20 and anti-IL6 have been used with varying success in various organ involvement. Other medications include: Tyrosine Kinase Inhibitor for lung fibrosis, Endothelin Receptor Antagonist (ERA) and PDE-5 Inhibitors for digital ulcers and pulmonary hypertension, Calcium Channel Blocker for Raynaud’s phenomenon, and Proton Pump Inhibitors for reflux.

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