Organs

Skin thickening is a common symptom in scleroderma, that is caused by scar tissue. In systemic sclerosis, this scar tissue or 'fibrosis' can become a very serious complication, as it forms within internal organs such as the lungs or the kidneys.

What causes the scarring?

In systemic sclerosis the immune system becomes overactive, causing the body to attack healthy tissues.

Part of the normal response of the immune system to deal with an injury or infection is to cause inflammation. When the immune system is overactive and continuously 'switched on', then this inflammation can cause damage to the body rather than healing it.

Normally, following an injury or infection, inflammation helps the body to repair itself by triggering the process of healing. For a more serious injury, part of the healing process involves scarring. In systemic sclerosis, excessive and continuous inflammation ultimately results in the development of scarring, or 'fibrosis'.

Kidneys

Although it is quite common for kidney function to be mildly reduced in scleroderma, this is not usually a major issue. If having scleroderma causes the kidneys to run at 50-70% of normal, this should still be sufficient for them to do their job adequately.

However, about 5-10% of people with systemic sclerosis (usually, but not only, those whose skin symptoms are worsening more quickly), will develop severe, uncontrolled high blood pressure from narrowed blood vessels within the kidneys. Over a short period of time, the narrowed blood vessels starve both kidneys of blood and oxygen, setting in motion the response of raised blood pressure. This eventually destroys the kidneys and damages the heart and lungs if left untreated. This process is called scleroderma renal crisis. The symptoms of scleroderma renal crisis are:

• • • • • •

Very high blood pressure

Severe headaches or blurred vision

Breathlessness

Nausea and vomiting

Heart palpitations

In some cases, seizures

Everyone with scleroderma renal crisis will initially need to be looked after in hospital. Some may require intensive care, coronary care or the specialist renal ward. Blood pressure medication will be given, and dialysis may be required if the kidneys have failed completely. People who do not recover after two years of dialysis may be candidates for a kidney transplant.

This English version is a translation; in case of doubt, the Bulgarian version applies.