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Scleroderma

What is Scleroderma?

Scleroderma is an autoimmune disease which affects skin and internal organs. The name scleroderma means “hard skin”. As the name implies the disease starts in the skin but goes on to involve other organs like kidney, lungs, heart and gastrointestinal tract. There are two types of scleroderma.  Diffuse and limited, which differ in the extent of skin involved.

Who gets scleroderma?

Scleroderma is a disease affecting approximately 25,000 patients in Kerala or 100,000 patients in India.  Of these, 75% percent are women, usually diagnosed between the ages of 30 and 50 years.

What causes scleroderma?

The cause of scleroderma is not known. Genetic factors appear to increase a patient’s chance of getting the disease. However, some data suggests that exposure to industrial solvents or an environmental agent may play a role in leading to scleroderma.
 
Clinical Features of scleroderma
Scleroderma is a multi system disorder affecting many systems in the body like,

Skin: Thickening and darkening of the skin

Blood vessels: Blood vessels shrink and become excessively sensitive to cold. Because of this the blood flow to extremities decreases and the skin of the hands and extremities becomes blue or pale on exposure to cold. This is colour change of the extremities is called Raynaud’s phenomenon. If not treated properly the blood supply decreases to such an extent that skin of the extremity becomes dark (dead) and very painful ulcer develops, which is very difficult to heal.

Gastrointestinal tract: Scleroderma affects every part of GIT from mouth to the anus. There will be difficulty in opening mouth in these patients. They also have burning in the chest due to excessive acidity. Also food can get stuck in the oesophagus. They can have diarrhoea or constipation, which vary from person to person.

Lungs: Lungs is one of the major organs which get affected in scleroderma. The symptoms of this will be cough and breathlessness. This is due to fibrosis in the lungs. If not treated early the fibrosis becomes very extensive and damages the whole lungs and can result in death of the patient.

Heart: Scleroderma can affect both sides, right and left, of the heart. This result in increase in pressure on the right side of heart called Pulmonary artery hypertension. On the left side it causes impaired pumping called left ventricular failure.

Kidney: Scleroderma can rarely involve kidney resulting in renal failure.

How is scleroderma diagnosed?

Scleroderma is diagnosed by clinical examination by a experienced clinician. Then the doctor has to do series of tests to see for the organ involvements in these patients including the pulmonary function tests (PFT), CT scan and Scan of the heart.

What are the treatment options available?

Scleroderma patients can be treated effectively with currently available dugs. The drugs used vary from person to person as the organs affected will be different in each one.

Raynaud’s phenomenon
Don’t smoke. Smoking narrows blood vessels, making Raynaud’s worse.
Avoid the cold when possible.
Dress warmly, in layers.
Use drugs prescribed by your doctor to  improve your circulation. (Calcium channel blockers, angiotensin receptor blockers, Tadalafil  etc).
Skin sores and ulcers can be treated with  nitroglycerine paste or antibiotic cream

Skin problems

Apply moisturizing creams and lotions frequently, especially after bathing.
Use only warm water in your bath or shower  (hot water is too drying)
Avoid harsh soaps, household cleaners and caustic chemicals.

Gastrointestinal problems
Eat small, frequent meals.
Take dinner before 8 pm
 Remain standing or sitting at least one hour after eating.
Avoid citrus fruits like orange and lemon at nights
.Proton pump inhibitors, such as omeprazole  and lansoprazole, can be taken for heartburn.

Lung disease
Immunosuppressive drugs such as cyclophosphamide and Mycophenolate Mofetil , along  with low-dose steroids can be used to treat  pulmonary fibrosis.

Heart disease
Vasodilators, such as prostacyclin, Bosentan, sildenafil can be used to decrease the pressure in the right side of the heart.

Can scleroderma patients lead a normal life?

If scleroderma is diagnosed and treated early it can be controlled very well. This is because in scleroderma most of the damage occurs early in the disease and if not controlled early the damage which happens is irreversible.