The Indian Rheumatology Association
The Professional Organization of Rheumatologists and other Health Professionals in India
Scleroderma or systemic Sclerosis

What is Scleroderma ?
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.

What causes it ?
The 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.

Symptoms

Localized Scleroderma
The symptoms of localized scleroderma are isolated ot the skin and underlying tissues. Two types are recognized: morphea and linear scleroderma.

Morphea : These are local patches of hardened skin. Red patches of skin develop white centers with purplish borders. Lesions remain active for weeks to several years. Spontaneous softening that leaves a darkened area of skin often occurs. It may be localized or generalized.

Linear: A single line or band of thickened and discolored skin develops. The line usually runs down and arm or leg, but sometimes it runs down the forehead.

Systemic Sclerosis
This disease affects not only the skin and underlying tissues, but also affects the blood vessels and major organs of the body. Two types of systemic disease are recognized: limited and diffuse.

Limited: In this form, sin thickening is generally limited to the fingers, forearms, legs, face and neck. Raynaud's phenomenon ( blue fingers ) may be present for years before any other symptoms develop. People with this form are less likely than people with diffuse disease to develop severe organ involvement.

Diffuse: In this form, skin thickening may occur anywhere on the body, including the trunk. Only a short interval of time will elapse between the onset of Raynaud's phenomenon and significant organ involvement. Damage typically occurs over the first three to five years, after which most patients enter a stable phase that varies in length. During this phase, your skin will stay about the same and the rate of damage to interval organs slows or stops. After the stable phase is over, your skin will start to soften and more serious damage to internal organs is unlikely to occur.

Depending on the form and severity of the disease, you may experience these different symptoms:

Raynaud's Phenomenon: Cold temperatures or emotional distress cause constriction of blood vessels in hands feet leading to cold extremities and whitish or bluish discoloration of fingers and toes.Once the vessels open back up ( after approximately 10 to 15 minutes), the hands turn red. More than 90% of people with scleroderma have this problem, it is often the first symptom of the disease. Skin ulcers on the fingertips can result from severe Raynaud's phenomenon.

Skin changes: In the early stage skin may appear edematous and it may progressively harden with loss of flexibility. As the disease progresses, the skin will become more thickened, sweat and oil glands will stop functioning, and the skin surface will become very dry and itchy. This stage will persist for one to three years, at which time the skin will begin to soften and thin.

Sclerodactyly: Thickening of the skin of the fingers or toes.

Calcinosis: Calcium deposits can form under the skin or in the muscles. These deposits may cause recurrent ulcers on the overlying skin.

Telangiectasias:Red spots due to dilated capillaries near the surface of the skin can be seen.

Arthralgias and myalgias: Pain and stiffness of the joints and muscles are common symptoms early in the course of systemic sclerosis. Later in the disease course, muscle atroply and weakness are common.

Dental disease: If the skin around your mouth tightens, you may not be able to open your mouth very wide.You may also have a dry mouth due to secondary Sjogren;s syndrome. Damage to connective tissues in the mouth can lead to loose teeth. These problems can lead to periodontal disease, tooth loss and difficulty chewing, which can compromise your nutritional status.

Esophageal dysmotility: difficulty in swallowing due to loss of normal movement in the food pipe. It may cause chronic heartburn

Gastrointestinal dysfunction: Dysmotility along the whole gastrointestinal tract can cause reflux, early satiety, bloating, nausea, vomiting, cramping abdominal pain, diarrhea, weight loss and malnutrition.

Lung disease: difficulty in breathing or sense of suffocation are the most common initial symptoms. Similar to skin thickening, lungs can become hard too and loose their elasticity leading to decreased oxygenation. Lung impairment is the leading cause of death in systemic sclerosis. Getting your lung function tested regularly is important for detecting lung disease early.

Kindney disease: sudden rise in blood pressure leading to kidney failure is a dangerous complication. About 80 percent of cases of renal crisis occur within the first four or five years of disease, usually in people with diffuse disease.

Heart disease: Symptoms of heart disease are not seen until late in the course of systemic sclerosis. Symptoms include breathlessness on exertion, palpitations and, less frequently, chest pain.

Is it a contagious disease?
No.

How to confirm the diagnosis?
A diagnosis could take months as the disease unfolds and as the doctor is able to rule out other possible causes of your symptoms. Diagnosis is made clinically by the doctor familiar with the disease. There is no direct blood test that can confirm the diagnosis. Special tests may be ordered to evaluate your lung status ( such as a chest X-ray, pulmonary function tests) and gastrointestinal tract function.

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.
See a physiotherapist to stretch contracted joints.

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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