Scleroderma or Systematic Sclerosis

Scleroderma literally means " hard skin," which is a common finding to this group of diseases. It accurs due to the abnormal growth of connective tissue. There are broadly two categories of scleroderma: localized scleroderma ( affects skin and rarely muscles ) and systemic sclerosis ( affects skin and internal organs like heart, lung, intestines etc).
Localized scleroderma generally remains limited and does not progress to the systemic form. Localized scleroderma further can two types; morphea and linear.
Systemic sclerosis affects the skin, muscles, blood vessels and internal organs. It is often divided into diffuse and limited disease. Diffuse systemic sclerosis is a rare disease that can be severe and sometimes life-threatening.

Scleroderma or Systematic Sclerosis
 
What causes it ?
he exact cause of scleroderma is not known. However, it has been known that body makes too much of collagen ( substance that heals and gives us scars ) that gets deposited in the skin and other body organs, causing the skin to tighten and harden and the organs to dysfunction. Another component is abnormal function of cells in the lining of blood vessels, which leads to Raynaud's phenomenon (pallor, blue fingers or toes) and telangiectasias (red spots). These abnormalities are triggered by abnormal functioning of our own immune cells, some environmental agents of hormones that stimulates cells to produce excess collagen.

What is the Treatment
No treatment till date has been proven effective in reducing the skin thickening. However, several treatments are very effective at preventing and/ or reducing organ damage from this disease. While treatments are not able to reverse the disease, they may actually prolong life. So treatment dicisions are made on a symptom-by-symptom, organ-by-organ basis.
 
Raynaud's phenomenon
Dont't smoke. Somoking narrows blood vessels, making Raynaud's worse.
 
Avoid the cold when possible.
Dress warmly, in layrs. Mitterns are warmer than gloves. Use drugs perscribed by your doctor to improve your circulation ( Calcium channel blockers, angiotensin receptor blockers, ACE inhibitors etc.) Skin sores and ulcers can be treated with nitroglycerin paste or antibiotic cream. Narcotic analgesics may be necessary to treat the pain of these ulcers.
 
Skin problems
  • Apply moisturizing creams and lotions frequently, especially after bathing. Apply sunscreen before going outdoors.
  • Use only warm water in your bath or shower ( hot water is too drying)
  • Avoid harsh soaps, household cleaners and caustic chemicals. Use rubber gloves if you cannot avoid harsh chemicals.
  • Itching can be treated with oral antihistamines, topical analgesics and topical corticosteroids
 
Stiff muscles and joints
  • Get regular exercise to maintain range of motion and muscle strength.
  • Use pain killers as prescribed by your doctor.
  • DO NOT mix up two or more NSAIDs on the same day.
  • DO NOT take pain killers empty stomach.
 
Dental problems
  • Practice good oral hygience- brush and floss regularly. Use fluoride rinses or prescription toothpastes.
  • See your dentist regularly.
  • Keep your mouth moist by taking frequent sips of water, chewing sugarless gum or using saliva substitutes.
  • Perform facial exercises to help keep your mouth and face flexible.
Gastrointestinal problems
  • Eat small, frequent meals.
  • Remian standing or sitting at least one hour after eating.
  • Avoid late-night meals.
  • Chew food well.
  • Proton pump inhibitors, such as omeprazole and lansoprazole, can be taken for heartburn.
  • Promotility agents may help move food through the gastrointestinal tract.
  • Antibiotics may relieve diarrhea caused by bacterial overgrowth.
  • Vitamin supplementation may be needed.
Lung disease
  • Immunosuppressive drugs such as cyclophosphamide and azathioprine, along with low-dose steroids can be used to treat pulmonary fibrosis.
  • Vasodilators such as prostacyclin, Bosentan, sildenafil can be used to treat pulmonary hypertension.
  • Get regular check-ups with your doctor and have lung function tests performed often so that lung disease can be caught early and treated.
  • Get flu and pneumonia vaccines.
 
Kidney disease
  • Measure BP regularly and alert your doctor if it is high. Angiotensin- Converting Enzymes (ACE) inhibitors, including captopril, enalapril and ramipril can effectively manage acute renal crisis by lowering high blood pressure.
  • Angiotensin II receptor inhibitors may be used to control hypertension when your are not in renal crisis.
  • Kidney dialysis may be necessary for some patients, atleast temporarily.
  • Renal transplantation may be necessary if kidney are damaged permanently.
Heart disease
Treatments for heart disease vary according to the way the heart is being affected by systemic sclerosis.
 
Who is at risk of getting this disease ?
  • Anyone can get scleroderma at any age. Women are more commonly diagnosed with scleroderma than men. In fact women with the disease outnumber men with it by about four-to-one.
  • Localized scleroderma is more common in children and young women.
  • Morphea usually appears between the ages of 20 and 40.
  • Linear scleroderma usually occurs in children or teenagers.Systemic sclerosis, whether limited or diffuse, typically occurs in people 30 to 50 years of age.
 
Life expectancy
Localized scleroderma is not life-threatening. Systemic sclerosis patients are at risk of serious complications, kidney disease, Pulmorary Arterial Hypertension (PAH) (high blood pressure in the arteries around the lugs), alveolitis ( inflammation of the lungs), Gastro-intestinal ( GI ) disease and heart damage that can be fatal and adversely affect the quality of life.
 
Pregnancy
Any female diagnosed with scleroderma needs to check with her physician and obstetrician to discuss possible complications associated with having children. If scleroderma has stabilized, then the physicians are better able to determine if the body can handle pregnancy and childbirth. The genetic risk of transmission of disease to children is negligible.
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