Systemic Sclerosis [SSc] or Scleroderma
Scleroderma IS ...
. A chronic, autoimmune disease of the connective tissue generally classified as one of the rheumatic diseases. Also known as systemic sclerosis.
. A disease in which the symptoms may either be visible, as when the skin is affected, or invisible, as when only internal organs are involved.
. A highly-individualised disease. Its involvement may range from very mild symptoms to life-threatening complications.
Scleroderma IS NOT ...
. Contagious
. Cancerous or considered malignant in any way
. Inherited, as a general rule
Who has Scleroderma?
. An estimated 10,000 persons in the United Kingdom have scleroderma.
. Approximately 4 times more women than men develop the disease.
Causes of Scleroderma
. The exact cause or causes of scleroderma are unknown. It is known that the disease is a complex one, which involves inflammation, blood vessel changes and fibrosis [scarring]. These symptoms can affect a number of organs including the heart, lungs, kidneys, gut and muscles.
Classifications of Scleroderma
Localised scleroderma - more common in children. Usually found in only a few places on the skin or in the muscles. Rarely, if ever, does localised scleroderma develop into the systemic form of the disease.
Systemic sclerosis May affect the connective tissue in many parts of the body, e.g. the skin, the oesophagus, gastrointestinal tract, lungs, kidneys, heart and other internal organs. It may also affect blood vessels, muscles and joints.
Symptoms of Scleroderma
May include one or more of the following
. Raynaud's Phenomenon [abnormal sensitivity to cold in the extremities]
. Swelling of the hands and feet
. Pain and stiffness of the joints
. Thickening of the skin
. Joint contractures
. Digestive system and gastrointestinal tract problems
. Sjogren's Syndrome [dry mucus membranes]
. Oral, facial and dental problems
. Kidney, heart and lung involvement
. Non-specific symptoms such as extreme fatigue, generalised weakness, weightloss and vague aching of muscles, joints and bones
How is Scleroderma diagnosed?
The diagnostic process may require consultations with rheumatologists [arthritis specialists] and dermatologists [skin specialists] as well as blood studies and a variety of specialised tests depending upon which organs are affected. The treatment program varies depending on the type and severity of the symptoms.
Diagnosis may be very difficult, particularly in the early stages, because many of the symptoms are common to, or may overlap with, those of other diseases, especially other connective tissue diseases such as rheumatoid arthritis, lupus and polymyositis.
Treatment of Scleroderma
Most treatment in scleroderma is directed to the relief of specific organ involvement.
Proton pump inhibitors are prescribed for oesophageal involvement that leas to heartburn and reflux.
ACE inhibitors are prescribed for renal failure and heart involvement leading to hypertension.
Pulmonary hypertension is treated with prostaglandin analogues to help dilate the blood vessels in the lungs. This treatment also benefits Raynaud's Phenomenon and ischaemic fingers and toes.
Pulmonary fibrosis is controlled by the use of drugs that modify the immune response to dampen down the activity, which is causing the damage.
Other drugs such as calcium channel blockers and angiotensin receptor antagonists are prescribed because studies have shown them to be beneficial sometimes in association with other treatment.
Specific anti-arrhythmics may be used for some cardiac conditions.
Therapies involving hot wax or skin massage are used to increase skin flexibility.
I have heard that scleroderma can be life-threatening
Patients with scleroderma are living longer than they did in the past, and dying from fewer scleroderma-related causes.
Years ago, renal failure in scleroderma invariably caused the death of the patient. It is now rare, since haemodialysis and ACE inhibitor treatment may allow sufficient kidney function to recover for the patient to live an almost normal life. Even if dialysis is prolonged, patients now survive total renal failure.
Pulmonary fibrosis, if unchecked, caused severe loss of mobility due to low exercise tolerance and susceptibility to chest infections. Fibrosis is now controlled early and patients stabilise with little permanent scarring. It is important to avoid smoking and to have 'flu' jabs in the autumn.
Infection following ischaemia of the fingers and toes is still a big problem. Some infections are resistant to multiple antibiotics and are very difficult to treat. Infection can lead to amputation of the limb, and on occasion septicaemia, which can prove fatal. Painkillers are also needed but opiates are not good since they depress the peripheral circulation. It is therefore important to attend to hygiene, and to have podiatric care of the feet on a regular basis, ischaemic attacks need to be attended to immediately. Smoking also brings on ischaemic attacks, and depressed the circulation.
The small bowel is also susceptible to ischaemia and infection, and also to 'pseudo-obstruction' when the bowel appears to be blocked.