Although over 90% of patients with Scleroderma have Raynaud's, the chances of someone with Raynaud's developing Scleroderma are small - it is less than 2% women and 6% in men. But the chances of someone with Scleroderma developing Raynaud's are pretty high.
A little about Scleroderma:
The word Scleroderma means hardening of the skin, although the condition is not limited to the skin. It is a disease of the connective tissue, which is the tissue that holds our bodies together.
Therefore, not only the skin can be affected, but also internal organs. The majority of sufferers have the mild form where there is limited skin involvement, usually of the hands and feet, becoming stiff and shiny. The gullet may also be affected making eating and swallowing difficult.
Some patients also form tiny deposits of calcium under the skin (calcinosis) which can cause ulcers. In the more severe form, called diffuse Scleroderma, wide areas of skin and internal organs such as the lungs, bowel heart and kidneys are affected. Localised Scleroderma can be divided into two types:
Morphea Scleroderma
Linear Scleroderma
How are these conditions diagnosed?
The history of the disease is most important. Blood tests may help, as can examining the small blood vessels at the base of the nail, which is called nail fold capillaroscopy.
The diagnosis of the Scleroderma is based on the finding of the clinical features of the illnesses. In addition, nearly all patients with Scleroderma have blood tests that suggest autoimmunity, because antinuclear antibodies (ANAs) are usually detectable. A particular antibody, the anticentromere antibody, is found almost exclusively in the limited, or CREST, form of scleroderma. Anti-Scl 70 antibody (antitopoisomerase-I antibody) is most often seen in patients with the diffuse form of scleroderma.
Other tests are used to evaluate the presence or extent of any internal disease. These may include upper and lower gastrointestinal tests to evaluate the bowels, chest X-rays, lung-function testing (pulmonary function test), and CAT scanning to examine the lungs, EKG and echocardiograms, and sometimes heart catheterization to evaluate the pressure in the arteries of the heart and lungs.
Is it hereditary?
There is no evidence at present that either Raynaud's or Scleroderma are directly inherited. There is however a genetic predisposition, so that the chances of being affected are greater if a relative has the problem.
The Treatment:
Just like the Rraynaud's, there is
no cure for Scleroderma at present but there are many effective treatments available to alleviate specific symptoms. As each case is different you should discuss these issues with your doctor.
Your General Physician or specialist may prescribe a vasodilator, which is a drug that relaxes the blood vessels. Occasionally, your specialist may feel an operation called a sympathectomy may be of benefit. This involves either cutting or destroying the nerves that cause the arteries to constrict. This operation is more successful for Raynaud's of the feet, however it is not recommended for the majority of cases as it does not usually produce longer term benefits.
People who develop
Raynaud's as teenagers often have a form that is benign and will disappear with age. Unfortunately this is not true in all cases, and sometimes Raynaud's does persist.
Treatment of scleroderma is directed toward the individual feature(s) affecting different areas of the body.
Aggressive treatments of elevations in blood pressure have been extremely important in preventing kidney failure. Blood-pressure medications, particularly the angiotensin converting enzyme (ACE) inhibitor class of drugs, such as captopril (Capoten), are frequently used.
Recent data indicate that colchicine can be helpful in decreasing the inflammation and tenderness that periodically accompanies the calcinosis nodules in the skin. Skin itching can be relieved with lotions (emollients) such as Eucerin and Lubriderm.
Mild Raynaud's phenomenon may require only hand warming and protection. Low-dose aspirin is often added to prevent tiny blood clots in the fingers, especially in patients with a history of fingertip ulcerations. Moderate Raynaud's phenomenon can be helped by medications that open up the arteries, such as nifedipine (Procardia, Adalat) and nicardipine (Cardene), or with topical nitroglycerin applied to the most affected digit (most effective on the sides of the digit where the arteries are). Gently applied finger splinting can protect tender tissues. (It is important to not constrict the tiny arteries on the sides of the fingers when protecting them with splints, braces, or band-aid materials.) A class of medications that is typically used for depression, called serotonin reuptake inhibitors, such as fluoxetine (Prozac), can sometimes improve the circulation of the affected digit. Severe Raynaud's phenomenon can require surgical procedures, such as those to interrupt the nerves of the finger that stimulate constriction of the blood vessels (digital sympathectomy). Ulcerations of the fingers can require topical or oral antibiotics.
Esophagus irritation and heartburn can be relieved with omeprazole (Prilosec), esomeprazole (Nexium), or lansoprazole (Prevacid). Antacids can also be helpful. Elevating the head of the bed can reduce the back-flow of acid into the esophagus that causes inflammation and heartburn. Avoiding caffeine and cigarette smoking also helps.
Constipation, cramping, and diarrhea is sometimes caused by bacteria that can be treated with tetracycline or erythromycin. Studies have shown that erythromycin could also be used. Increased fluid intake and fiber intake are good general measures.
Irritated, itchy dry skin can be helped by emollients such as Lubriderm, Eucerin, Bag Balm histamine 2 blockers, or trazodone (Desyrel).
Telangiectasias, such as those on the face, can be treated with local laser therapy. Sun exposure should be minimized as it can worsen telangiectasias.
Approximately 10% of patients with the CREST variant develop elevated pressures in the blood vessels to the lungs (pulmonary hypertension). Abnormally elevated blood pressure of the arteries supplying the lungs is often treated with calcium antagonist medications, such as nifedipine (Procardia), and blood-thinning drugs (anticoagulation). More severe pulmonary hypertension can be helped by continuous intravenous infusion or inhalation of prostacyclin (Iloprost). Taken by mouth, bosentan (Tracleer), is now also available to treat pulmonary hypertension. In addition, sildenafil (Viagra) and tadalafil (Cialis) have been FDA approved to treat pulmonary hypertension.
Additionally, medications are used to suppress the overly active immune system that seems to be spontaneously causing the disease in organs. Medications used for this purpose include penicillamine, azathioprine (Imuran, Azasan), and methotrexate (Rheumatrex, Trexall). Recent research has found that low-dose penicillamine (Depen, Cuprimine) (125 mg every other day) is as effective as previously used high doses of penicillamine, with less toxicity. Serious inflammation of the lungs (alveolitis) can require immune suppression with cyclophosphamide (Cytoxan) along with prednisone (Deltasone, Liquid Pred). The optimal treatment of scleroderma lung disease is an area of active research. Stem-cell transplantation is being explored as a possible option.
No medication has been found to be universally effective for all patients with scleroderma. In an individual patient, the illness may be mild and not require treatments. In some, the disease is ravaging and relentless.
One can find more info about medications for Scleroderma on following website:
http://www.hopkinsscleroderma.org/patients/scleroderma-treatment-options/
Precautions:
There are several things you can do which may help. The most important is to stop smoking, take regular exercise and keep warm.
Most of the
precautions are same as with Raynaud's.
Here are the
Do's For the Patient's of Raynaud's Phenomenon.
And these are the
Don'ts For the Patients of Raynaud's Phenomenon.
Smoking
If you are a smoker you must make a sincere and determined effort to give up completely. Tobacco is harmful as it causes the blood vessels to constrict, decreasing the blood flow to the fingertips. Your GP should be able to discuss strategies for you to give up smoking or arrange for you to see a smoking cessation counsellor. Nicotine replacement may also help and you should discuss this with your doctor or pharmacist.
One can find more detailed information of smoking on human body here:
Raynaud's Phenomenon and Smoking
Eating for warmth
Eating and drinking can help you keep warm. Try to eat lots of small meals to maintain your energy; high protein foods, milk, meat, fish, and fresh vegetables are best. Hot meals and plenty of hot drinks are essential.
(I am currently working on a post
completely about diet for Raynaud's, I will publish it as soon as possible.)
Exercise
Gentle exercise will help your circulation. Try to avoid sitting for long periods. Get up and walk around the room, moving arms and legs to maintain the circulation. Do not however let your fingers or toes get cold. In cold weather take exercise indoors.
Clothing
Tight clothing should be avoided as this may restrict blood flow. Hands and feet should always be adequately covered. A scarf should be used to keep the face warm in cold weather.
One should wear a hat, because most of the body heat gets released from the head.
So, keep the head and trunk warm, because the
core temperature of the body is the thing which really matters.
Feet are especially prone to cooling, therefore a good thick pair of socks is essential. Wet shoes and clothes should be changed as soon as possible.
One can find more information about clothing, skin protection, and the effect of alcohol regarding Raynaud's in the index.
Warm Regards,
Kiran Nayak :-)
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