Scleroderma is a complex disease with many possible symptoms that can affect many parts of the body. Everyone with Scleroderma has different symptoms and severity. Usually symptoms can also vary over time with periods of improving and worsening. Although Scleroderma can not be cured, most of the symptoms may improve with medication, as well as with lifestyle changes. Not all ‘people with Scleroderma’ will have all the symptoms as listed below:
· Thickening of the Skin
Hardening and thickening of the skin is the basic of the name Scleroderma (“sclero: hard”). There has been no proven treatment to prevent or even alter the course of skin changes in Scleroderma.
· Skin Wounds (Ulcer)
The ulcer that occurs is a common phenomenon of Systemic Scleroderma. Injuries occur in the knuckles, the tips of the fingers and toes, elbows or other body parts where the skin becomes very tight/stretched. The ulcer is a result of the Raynaud phenomenon (which is described below). The affected area should be kept warm to increase blood flow and oxygen, and be kept completely clean to avoid infection. To heal the wound/ulcer will take a long time (4-7 months) due to poor blood vessel circulation.
If this treatment is proven to not reduce pain or ulceration of skin infections, your doctor will usually prescribe oral antibiotics or take other actions. It is important to consult with your doctor as early as possible if you suspect there are infected wounds, because early treatment can prevent further damage. If you have an ulcer that does not heal, you may want to discuss it with your doctor to be given a referral to the vascular surgery center to get help wrapping and treating ulcer.
This condition is characterized by the presence of calcium deposits in the skin, which is sometimes painful. Calcium deposits may occur just beneath the surface of the skin in the form of a hard lump or nodule. The sediment can penetrate the skin, so it looks with the eyes like a white material like chalk, and can become an infection. Treatment should be done by soaking in warm water. Usually the doctor will prescribe antibiotics to help prevent or control the infection. In severe cases, surgical operations to remove/remove calcium, may be necessary.
This disorder is a dilation of small blood vessels on the surface of the skin, which looks like small red spots, usually in the fingers, palms, face and lips. The spots usually fade when pressed, but become red again when the pressure is released. Such spots are usually harmless. Special cosmetics can be used to cover these spots. Laser therapy by trained physicians can also be done to reduce the number of telangiectasia, although it is rare to find telangiectasia that grow back in treated areas.
· Dry Skin
Excessive dryness of the skin can cause skin damage and ulceration. Avoid bathing and washing hands too often, avoid and use rubber gloves to avoid direct contact with household detergent products. To avoid complications from dry skin it is recommended to keep skin moist and lubricated with body cream or olive oil. Bath oils and moisturizing soaps like Neutrogena, Dove, or baby soap are more advisable than hard soaps that can dry the skin. It is also recommended to frequently use a skin moisturizing cream containing lanolin or sorbelene.
· Itchy Skin
If the moisturizing cream can not reduce the itch, your doctor may prescribe topical cortisone cream to be rubbed onto the skin that serves to relieve itching. An antihistamine tablets proved to be effective for some people.
· Other Skin Symptoms
Reduced hair around affected skin, decreased ability to sweat. Skin redness when exposed to sunlight. In addition, there is an increase in pigment that looks like white patches spotted brown because the skin loses pigment/dye conditions is called salt and paper. Hair loss.
· Sclerodactyly and Joint Inventions
Sclerodactyly marked with shiny and tight/fingered fingers. The affected fingers are difficult to move, and being stiff in a bent or bending position, the position is called contracture or flexion contractures. The hardened and rigid skin of the joints can lead to a decrease in the function of other wrist, elbow and joint movements. The range of motion exercises are performed every day to prevent or slow the progression of joint contractures and keep the joints flexible. It can also help increase blood flow to the tissues. A typical exercise is to put the hand as flat as possible on the table, place the other hand on the fingers at an angle, and press down slowly and gently to straighten the fingers. An expert therapist can develop an exercise plan after consulting your doctor. He can also provide tools to help you to take care of yourself and do housework easier.
Pain and stiffness in the joints
The symptoms of pain, stiffness, swelling, accompanied by arthritis such as arthritis often occur in Scleroderma. Anti-inflammatory/anti-inflammatory medications can help to relieve pain in these conditions.
Swelling is one of the early typical symptoms of scleroderma that arise when waking up due to muscle inactivity at night. The skin of the fingers looks solid like a sausage, so the hands are hard to fist.
Raynaud’s phenomenon is the most common early symptom of Systemic scleroderma. This symptom is present in approximately 90% of patients. Most noticeable on the fingers, feet, ears, nose and tip of the tongue. In Raynaud’s phenomenon, blood vessels narrow when exposed to cold temperatures or emotional states such as fear/anxiety/stress. Blood circulation disorders cause skin discoloration to white, paled, to blue. This condition occurs when the blood and oxygen flow in the blood vessels is not smooth. At the condition of the fingertips and feet will experience tingling, numbness, numbness and cold. In some patients, Raynaud’s phenomenon is a painful symptom. Some precautions that can be done by patients who are vulnerable to Raynaud’s phenomenon, among others:
1. Avoid cold, such as outdoor weather/air conditioner, keep body temperature warm, wear jacket, gloves, socks, scarf, use of heating pad or hot water bottle at the back of body/back and other heating fixtures . It is important to protect the hands with gloves when touching food or ingredients that are refrigerated or frozen in the refrigerator.
2. Bathing with warm water, using footwear inside the house, minimize exposure to water; using rubber gloves when having to do housework such as washing dishes/ washing clothes.
3. Avoiding stress; various relaxation techniques have proved effective for some people in managing stress.
4. Not recommended smoking.
5. When the Raynaud phenomenon occurs, waving in a circular motion with caution can help to restore blood circulation. Massaging the hands and feet can also help.
Your doctor will suggest drugs to prevent, reduce the frequency or minimize the impact of Raynaud’s phenomenon. Most of these drugs are to dilate or open blood vessels. The drugs most commonly used and most tolerated are calcium channel antagonists, for example Adalat, Norvasc, Felodur and Diltiazem. ACE inhibitors can also help, Fluoxetene and antidepressants used to treat Raynaud have a good effect. However, these medications can cause side effects such as heart palpitations, facial flushing, headache, light dizziness, swollen ankles and constipation.
All side effects only temporarily and will disappear once the drug is stopped or the dose is reduced. In severe cases, especially when there is digital ulcer (an open wound on the fingertip) or infection, treatment with prostaglandins or prostacyclin infusions is usually recommended. This treatment is given by droplets into the blood vessels (intravenous). The duration of treatment is usually 3 days but may vary depending on the condition. This treatment can improve improvements in Raynaud for three months or more and has been shown to help digital healing ulcer in some people.
Raynaud’s phenomenon is not limited to people with Scleroderma. It is also seen in Lupus, Rheumatoid Arthritis, and other autoimmune diseases.
Sclerodactyly and Joint Contractures
Sclerodactyly marked with shiny and tight/fingered fingers. The affected fingers are difficult to move, and being stiff in a bent or bending position, the position is called contracture or flexion contractures. The hardened and rigid skin of the joints can lead to a decrease in the function of other wrist, elbow and joint movements. The range of motion exercises are performed every day to prevent or slow the progression of joint contractures and keep the joints flexible. It can also help increase blood flow to the tissues. Exercise is to put the hand as flat as possible on the table, and press down slowly and gently to straighten the fingers. An expert therapist can develop an exercise plan after consulting your doctor. He can also provide tools to help you to take care of yourself and do housework easier.
Digestive System and Digestive Tract Problems
In people with Systemic Scleroderma can have digestive and digestive system disorders from the mouth to the anus. The overproduction of collagen is a hallmark of Scleroderma that can cause thickening and fibrosis (scarring). This can cause the muscles to become weak, and the swallowing movements become abnormally slow (dysmotility) in the digestive process.
· Functional Abnormalities in the Esophagus
Food runs from the mouth and throat into the stomach through a tube called the esophagus. Usually, the lower throat sphincter or valve acts as a gate that will open and let food into the stomach then closes quickly to prevent food back up. In Systemic Scleroderma, the gate does not close properly and the result is a rise in stomach acid and a sense of heat (heartburn) as food and acid return to the esophagus. This acid can also injure the bottom layer of the esophagus, which can cause scarring and narrowing of the tube.
Stomach acid production in the stomach can be reduced by avoiding alcohol, fatty or oily foods, spicy foods, chocolate, tobacco and caffeine.
Upright position after meal is helpful for storing food and acid in the stomach, or eating smaller and more frequent meals, not eating for several hours before bedtime, and raising the head of the bed six to eight inches higher.
· Difficult to Swallow
Abnormally slow food movements and narrowing of the esophagus can cause difficulty in swallowing. It is recommended to eat slowly and chew well. If you need to eat softer foods (mashed with a blender) and avoid foods that tend to stick in the throat. If the esophagus has narrowed significantly, your doctor will suggest dilating the esophagus regularly to make it easier to swallow food.
In Systemic Scleroderma, there is a risk of damage to the muscles of the small intestine (small intestine). Weakened muscles do not work effectively to push food through the intestine. One result is excessive bacterial growth, which causes diarrhea. There is also a feeling of bloating, distended stomach and pain if the intestines lie. Another effect is the nutrients of food left in the intestine and not absorbed into the body. This condition is called malabsorption, and can cause drastic weight loss and stool/faecal abnormalities. Usually your doctor will advise to reduce the amount of fatty foods and increase the amount of carbohydrates or advise you to consult a nutritionist.
Muscles that are weak and injured in the colon wall will make the intestine difficult to work properly, resulting in constipation or other bowel abnormalities. Diet with high fiber, drinking water at least 6-8 glasses per day, eating fresh fruits and vegetables as a natural laxative, and moderate exercise regularly will help reduce constipation.
· Mouth, Face and Teeth
People with Scleroderma will experience skin tightening, decreased ability to open the mouth (microstomia/small mouth) which will affect the oral and dental hygiene. The best approach is to practice facial stretching and mouth, such as grimacing, A I U E O, frowning. Good and regular dental care by brushing your teeth and gums after meals. visiting a dentist/oral surgeon on a regular basis is also important to help prevent tooth decay.
Kidney involvement in Systemic scleroderma can occur from very mild to very severe. Early signs of kidney involvement include; mild blood pressure (hypertension), protein in the urine and blood test abnormalities. Kidney crisis can occur suddenly, this complication is very dangerous on Scleroderma Siytemic. The most important signal is the rise in blood pressure. Kidney crisis occurs in about 10% of people with Scleroderma Spread and commonly occurs in the first four years of their illness. Very rarely found kidney crisis in people with Scleroderma Limited.
Other symptoms that may occur are severe headache, vision disorders, shortness of breath, chest pain, and mental confusion. If not treated promptly, a kidney crisis can be a kidney failure, a condition in which the kidneys lose the ability to remove waste products from the body. People with scleroderma are advised to monitor / monitor their blood pressure and kidney function on a regular basis. Kidney crisis can improve, if the problem is known and treated quickly.
In Scleroderma, the lungs can be affected through three different processes:
· Interstitial Lung Disease (ILD)
Collagen accumulation that makes the lung tissue thickens and causes fibrosis or scarring, making the flow of oxygen in the blood more difficult. This is called pulmonary fibrosis or interstitial lung disease (ILD). Interstitial lung disease in people with Scleroderma can range from very mild and not progressive to severe and widespread or progressive conditions. Fibrosis of the lungs occurs up to 50% in people with scleroderma. Only a few people with elevated conditions and have a big impact on their lungs. Studies show that people whose lungs have been affected can be severe, most likely a significant decrease in lung function within the first four years of their scleroderma disease.
In the early years of diagnosis your doctor will suggest a lung function test in order to detect whether or not lung involvement is involved. If any involvement is detected in a lung function test, your doctor may also recommend a chest CT scan to determine also whether there is evidence of Interstitial Lung Disease/ILD. Recent studies have shown that patients with severe and progressive pulmonary fibrosis may benefit from some immunosuppressant drugs such as Cyclophosphamide.
· Pulmonary Arterial Hypertension (PAH)
Pulmonary Arterial Hypertension is a condition in which there is increased resistance to blood flow through the lungs and can cause damage to the blood vessels. This can lead to increased heart tension resulting in heart failure. PAH develops in 10-15% of patients with Systemic Scleroderma. PAHs often occur after several years of scleroderma. Pulmonary arterial hypertension (PAH) can now be treated with a specific drug intended to dilate or open the lung blood vessels. PAH is one of the complications of Scleroderma that has proven successful with new drugs.
There are three different types of drugs used to treat PAHs. The most commonly used is the enodothelin antagonist. This drug blocks the action of natural cytokines from endothelin that naturally increase cytokines in patients with PAHs. Endothelin is a very powerful constrictor of blood vessels in the lungs and serves to increase blood flow through the lungs. There are currently three different types of endothelin antagonists; Bosentan, Sitaxentan and Ambrisentan, Iloprost is inhaled through the nebulizer used every 2-3 hours during the day, and Sildenafil. All of these drugs have been shown to improve the quality of life and life expectancy of people with PAHs. These medications may be prescribed only by specialists with experience in PAH management and require careful and regular monitoring.
Early detection and treatment of PAHs can show results for those affected by this condition. To find out the presence of PAH your doctor will recommend performing lung function and cardiac ultrasound (Echocardiography) tests This test can be used to detect early stages of PAH.
· Chest Wall Involvement
Weakened breathing muscles and tightened chest wall can reduce lung function and cause breathing difficulties.
Symptoms of lung involvement include shortness of breath, decreased tolerance to exercise and persistent coughing. If you have any of these symptoms, your doctor will recommend an X-ray or chest CT scan, echocardiography (a cardiac ultrasound) and a special respiratory test (lung function test) to detect lung involvement and determine the symptoms which of these three processes. However, as mentioned above because of new therapies for pulmonary complications, Scleroderma specialists will increasingly recommend routinely screening for these complications with pulmonary function and echocardiography, even in people with no symptoms of lung problems. People with Scleroderma should be able to control the disease and avoid further lung damage by avoiding smoking and exposure to air pollution.
If the heart muscle thickens and scar tissue builds up, the strength of the heart’s contraction will decrease, which can eventually lead to heart failure. Coronary arteries (the main blood vessels to the heart) that seizures can cause chest pain but rarely lead to heart attacks. Seizures that appear similar to those occurring on the fingers in Raynaud’s phenomenon. Inflammation of the outer lining of the heart (pericarditis) can cause pain and fluid accumulation around the heart. Irregular heartbeat may also be experienced. This condition requires careful evaluation and treatment by specialists.
People with Systemic Scleroderma may experience a variety of non-specific symptoms, including fatigue (ranging from mild to severe or called fatigue), lack of energy, general weakness, weight loss, and muscle, joint, or bone pain. The treatment or treatment recommended by your doctor will depend on the results of the evaluation of the cause of this symptom.
The symptoms of Scleroderma vary from person to person, handling requires different treatment.