• What is systemic sclerosis (scleroderma)?

    Scleroderma is a long-term condition that causes thickening and hardening of the skin. Scleroderma is the older name for systemic sclerosis. It mostly affects the skin but it can also affect other parts of the body (joints, muscles, blood vessels, lungs, kidneys and digestive system) in addition to the skin. The word 'systemic' refers to a disease/condition that can affect different organs or systems of the body. ‘Sclerosis’ refers to hardening. Hence the name systemic sclerosis is preferred.

  • What are the symptoms of systemic sclerosis?

    Most common symptoms of the systemic sclerosis are:

    Changes in the skin:

    The most common changes in the skin include:

    • Thickening and hardening of the skin on hands, arms and face
    • Swelling of the hands and/or feet, especially in the morning
    • Shiny skin, without its usual creases
    • Stiffness of the facial skin, making it difficult to open the mouth wide, and sometimes thinning of the lips
    • Small red blood spots (called telangiectasia) on the face, hands and face

    Less frequently, or later on in the condition, there may be:

    • Thinning of the pads at the finger tips and the soles of the feet
    • Peeling, cracking or open sores (ulcers) in the skin and flesh over the fingertips, caused by poor blood supply
    • White chalky lumps under the skin (calcinosis), caused by calcium-containing deposits, often on the fingertips

    Increased sensitivity to the cold

    Fingers turn white and then blue in the cold (Raynaud’s phenomenon). This can happen even by just walking into a cold room or reaching into the fridge. It’s possible to have Raynaud’s without systemic sclerosis, but most people with systemic sclerosis will have symptoms of Raynaud’s at some time during their illness, and it’s often one of the first symptoms to appear. It can sometimes appear years before the onset of systemic sclerosis.

    Joint or muscle pain and stiffness

    systemic sclerosis can cause the tissues around the joints to stiffen, which makes the joints contract (bent position, called contractures). It can lead to:

    • Joint inflammation
    • Pain
    • Stiffness
    • Swelling
    • Tenderness

    Muscle weakness (myositis) is also sometimes a symptom of systemic sclerosis. About 1 in 5 people with systemic sclerosis will also have symptoms of a second rheumatic condition such as rheumatoid arthritis, lupus or Sjögren’s syndrome.

    Digestive problems

    Difficulty swallowing, heartburn/reflux

  • Who gets systemic sclerosis?

    Systemic sclerosis is rare disease. It usually starts between the ages of 25–55 and only occasionally begins in children or in older people. Women are 3–4 times more likely to develop it than men.

  • What causes systemic sclerosis?

    Systemic sclerosis is an autoimmune disease – a condition in which our own body’s defense system (immune system) becomes overactive and produces antibodies that fight against one’s own body tissues. It mostly affects the skin, but it can also affect other parts of the body viz., under the surface of the skin, around the internal organs and blood vessels. During which too much fibrous tissue is developed, which makes body’s tissues stiffen and thicken by holding them together. Although, the exact reason is not known yet, a mix of genetic and environmental factors are believed to play a part.

    Although we inherit these genes from our parents, the risk of developing systemic sclerosis is not passed directly from one generation to another. Because systemic sclerosis is not a contagious disease, it cannot be caught from somebody else.

  • How is systemic sclerosis diagnosed?

    There is no single test for systemic sclerosis, and the characteristic thickening of the skin is often the key factor in making the diagnosis. However, tests can be helpful out whether other parts of the body are affected. Tests could include:

    • Blood tests
    • X-rays and computerized tomography (CT) scans
    • Breathing tests
    • A heart scan (echocardiogram or ECG)
    • Stomach tests (for example and endoscopy)
    • A skin biopsy, where a small piece of skin is removed and examined under a microscope

    You may also have a capillaroscopy, which looks at the small blood vessels (capillaries) under a microscope, or thermography, which takes images of the heat coming from your body using an infrared camera, although they are often only performed at specialist centers.

    After you have been diagnosed, you might need to see your doctor regularly to monitor the condition. Most people have yearly test to check for early signs of the more serious complications, such as effects on the heart and lungs.

  • What treatments are there for systemic sclerosis?

    At present there is no cure for systemic sclerosis, although medications can help to control the symptoms and treat any complications. Based on the symptoms and complications the drugs are as follows:

    Treatment for Raynaud’s phenomenon:

    • There are quite a few drugs viz., nefedipine tablets that relax the blood vessels and there by improve the blood circulation to the hands and feet.
    • They are effective if more than one is used at the same time
    • In more severe cases of Raynaud’s, where the ulcers won’t heal, drugs such as iloprost can be given through iv infusion. This may require hospitalization
    • These drugs may cause side effects such as flushing, headache, swollen ankles

    Treatment for heartburn (reflux) and swallowing difficulties:

    • For this your doctor may recommend antacids or a drug that lowers acid production in the stomach. You may need to take them for a much longer period than is usually with simple heartburn. Your doctor may also prescribe a drug called as a proton pump inhibitor (PPI), which will help to protect your stomach

      Other gastrointestinal problems:

      • You may need to see a gastroenterologist if you have: spells of constipation and diarrhea, abdominal swelling or increased wind and discomfort. Some people may experience anal inconsistency, meaning they cannot control their bowel motions properly so that small amounts of stool slip out and can soil their clothes.
      • Sometimes medicines such as antibiotics may be given to control the additional bacteria developed in your bowel.

    Treatment for inflamed joints:

    Painkillers and non-steroidal anti-inflammatory drugs (NSAIDs) should help to relieve joint pain and inflammation. Like all other drugs NSAIDs have side effects such as stomach upsets, indigestion, damage to stomach linings. So in most cases they are given with a PPI. Even with PPI they are only given for a short period. NSAIDs also carry the increased risk of heart attacks or stroke. So your doctor will be cautious about prescribing NSAIDs if you have other risk factors viz., smoking, circulation problems, high blood pressure, high cholesterol or diabetes.

    Treatment for high blood pressure and kidney complications:

    High blood pressure sometimes occurs in systemic sclerosis and in severe cases this can lead to kidney damage and strain on the heart. This is a serious complication known as systemic sclerosis renal crisis. It can be treated or often prevented with drugs that help to control the blood pressure, especially ACE inhibitors.

    Treatment for lung and heart complications:

    Inflammation of lungs can be treated with steroids or disease-modifying anti-rheumatic drugs (DMARDs). High blood pressure in the lungs (pulmonary hypertension) is a rare complication in systemic sclerosis, but you will have regular tests to check how well your lungs and heart are working, which will pick up any problems. If necessary, pulmonary hypertension can be treated with specific drugs (including baseman, ambrisentan, sildenafil or iloprost) that improve symptoms such as breathlessness.

    Other drugs that may be used include:

    • Steroid tablets: they may be used either in the early stages of the disease when the skin is just starting to look puffy or later on if the muscles or lungs are effected. They are usually given in low doses because high doses can raise blood pressure and increase the risk of kidney problems
    • Immunosuppressive drugs:they target your immune system and may be used in more severe cases of systemic sclerosis, especially where the skin or lung disease is more extensive
    • Drugs to treat blood pressure and cholesterol

    Apart from the drugs mentioned above, there is a lot that you can do for yourself to help with the symptoms of systemic sclerosis viz., physical activity/exercise and eating a well-balanced diet will help ease the symptoms of systemic sclerosis.

  • Self-help and daily living

    Although drugs are important in controlling systemic sclerosis, there’s a lot that you can do to help manage your symptoms.

    • Following a healthy diet
    • Regular exercising
    • Dressing to keep your hands and feet warm in cold weather
    • Skin care

    Exercise:

    • This will keep your skin flexible
    • Reduce any tightening in your joints and keep them moving as much as possible
    • keep your blood flowing freely
    • stretching exercises should be done regularly, as this should help reduce joint contractures
    • a physiotherapist would help suggest the best exercise for joint mobility or facial mobility and splints (for managing hand contractures)

    Diet and nutrition:

    Maintaining a healthy diet is important as it may help Raynaud’s symptoms and to heal the skin ulcers. However, it is difficult to keep up a balanced diet and to keep your normal weight when you have digestive problems, heartburn or difficulty in swallowing. Below tips may be helpful in such conditions:

    • Eat six small meals in a day instead of three large meals
    • Eat slowly, chew thoroughly and drink plenty of water with meals
    • Taking your largest meal in the middle of the day can help to avoid heartburn. If you still face the heartburn consult your doctor
    • Don’t eat too much in the evening to make sure you have enough time for digestion before you go to bed.
    • Raise the head of your bed a few inches to stop acid coming back up from your stomach into your gullet while you sleep.

    If you find difficult to fit in six small meals, you can eat a healthy snack instead. The key thing to be aware of is that you are getting all the nutrients you need and don’t feel hungry. Sometimes you may need additional nutritional supplements.

    Skin care and keeping warm:

    You need a good supply of blood flowing to your skin to stop it from cracking, peeling and developing ulcers.

    • Keep warm from top to toe this will help open the blood vessels to your arms, hands, legs and feet. Wear a hat to help preserve your body heat. Thermal clothes, hand warmers and electrically heated gloves and socks can also help. Remember that layers of clothing will trap heat and keep you warmer than thick clothes.
    • If your skin is broken or painful, dressings can help to protect it
    • Don't use strong detergents or anything else that irritates your skin, and avoid soaps that contain lanolin. Try soaps, creams and bath oils designed to prevent dry skin until you find the ones that give you the best results in keeping your skin supple
    • If your hands are prone to dry skin, put cream on them whenever they have been in water. You can use either a water-based cream (such as E45 or aqueous cream), which is short-acting, or an oil-based cream (such as emulsifying ointment), which is thicker and longer lasting
    • Smoking reduces the blood flow to your skin and is very likely to make Raynaud's symptoms worse, so it's best to Stop
  • Complementary medicine

    Massaging the hands using warm paraffin wax can help in keeping the skin flexible and reducing joint discomfort, although you shouldn’t use a wax bath if you have any open finger ulcers. Aside from this there is no scientific evidence that any form of complementary medicine can help ease the symptoms of systemic sclerosis.

    Some people with Raynaud’s find that taking vitamins can help to control symptoms. The use of high dose of vitamin E and C, fish oil and ginger or gingko dietary supplements can also help. If the vitamins do not help within a three-month period you should stop takin them, but always consult your doctor before you try anything.

    Generally speaking, complementary and alternative therapies are relatively well tolerated if you want to try them, but you should always discuss their use with your doctor before starting treatment. There are some risks associated with specific therapies.

    In many cases, the risks associated with complementary and alternative therapies are more to do with the therapist than the therapy. This is why it’s important to go to a legally registered therapist, or one who has a set ethical code and is fully insured.

    If you decide to try therapies or supplements, you should be critical of what they’re doing for you, and base your decision to continue on whether you notice any improvement.

  • Supports, aids and gadgets

    If you have trouble with daily activities like dressing or tasks that need you to have good grip strength, there are a number of gadgets available that can help. If in doubt, ask an occupational therapist for advice on how to protect your joints from unnecessary strain.

    If you struggle to open childproof medicine containers, ask your pharmacist to put your drugs in containers you can manage. Some people find it difficult handling coins when their fingers are sore or swollen. A coin purse that opens out to form a tray for the coins may help with this.

  • Dealing with stress

    There may be emotional difficulties connected with having a long-term condition, and the changes in the appearance of your skin can be upsetting. In addition, stress can reduce the blood flow to some parts of your body, so it can affect your condition, particularly if you have Raynaud's phenomenon.

    Talk about any feelings of stress or depression with your family, friends or a healthcare professional. If you need help in handling stress or depression, your doctor may be able to help or can refer you for specialist counselling. You can also speak to a nurse in the rheumatology clinic — many clinics have nurses who either specialize in systemic sclerosis or have a special interest in the disease — or get in touch with a systemic sclerosis society, where you can talk with people who have the same condition.

  • What is the outlook?

    Systemic sclerosis is different for everyone, so it's hard to say how it might affect you. Most people find that systemic sclerosis affects just a few parts of the body and comes on gradually. It may slowly get worse but usually settles down to become stable after a few years. Sometimes systemic sclerosis may progress more quickly, but other people find that it almost disappears after several years.

    For some people the skin symptoms are the most troublesome, while others are affected more by poor circulation or digestive problems. Some people find their symptoms improve in summer but become worse in the winter.

    Some people have more serious complications, for example:

    • Scarring (fibrosis) of the lungs, causing shortness of breath and/or a dry cough
    • Narrowing of the blood vessels of the lungs, leading to high blood pressure in the lungs (pulmonary hypertension), which may in turn strain the heart
    • Kidney problems resulting in high blood pressure.

    These complications are fairly rare, but regular check-ups, preferably yearly, are recommended so that any problems can be spotted at an early stage.

    Because the body has the ability to break down or repair extra scar tissue, there may be some improvement in scarring — both in the skin and in other organs — once the disease becomes less active.