What is Systemic Sclerosis?
Systemic Sclerosis or Scleroderma is a multisystem autoimmune disease characterized by
vasculopathy (abnormal blood vessels), fibrosis of the skin and internal organs commonly
the lungs, heart and gut. The condition is divided into 2 main types “Diffuse” and “Limited”
depending on the extend 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 fingers
to below the elbows or from toes to below the knees. Both types can have facial skin
thickening. Limited type is more likely to have heart involvement while diffuse type more
likely to have lungs 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 will be thickening of skin, joints stiffness and pain, mouth
becoming smaller, reflux, heart burn, difficulty in swallowing, red rashes over face, neck or
hands. In more chronic or advance cases, patient may have painful ulcers at the fingers or
toes, cough and shortness of breath when walking or at rest, abdominal bloatedness,
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 extend 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 Methotrexate, Cyclophosphamide,
Mycophenolate, Rituximab, Tocilizumab have been used with varying success in
various organs involvement. Other medications such as nintedanib for lung fibrosis,
Endothelin Receptor antagonist (ERA) (Sildenafil, Tadanafil) and PDE-5 inhibitors
(Bosentan, Macitentan, Ambrisentan) for digital ulcers and pulmonary hypertension,
calcium channel blocker for Raynaud’s phenomenon and proton pump inhibitors for
reflux.
