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.

The cause of scleroderma is not known. Genetic factors appear to increase a patient’s chance of getting the disease. However, some data suggest 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 color change of the extremities call Raynaud’s phenomenon. If not treated properly the blood supply decreases to such an extent that the 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 the GIT from the 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 esophagus. They can have diarrhea or constipation, which vary from person to person.

Lungs: Lungs are one of the major organs which gets affected in scleroderma. The symptoms of this will be coughing 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 an increase in pressure on the right side of the 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.

Scleroderma is diagnosed by clinical examination by an experienced clinician. Then the doctor has to do a 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.

Scleroderma patients can be treated effectively with currently available drugs. 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.

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.