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.
Most common symptoms of the systemic sclerosis are:
The most common changes in the skin include:
Less frequently, or later on in the condition, there may be:
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.
systemic sclerosis can cause the tissues around the joints to stiffen, which makes the joints contract (bent position, called contractures). It can lead to:
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.
Difficulty swallowing, heartburn/reflux
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.
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.
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:
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.
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:
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.
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.
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.
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.
Although drugs are important in controlling systemic sclerosis, there’s a lot that you can do to help manage your symptoms.
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:
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.
You need a good supply of blood flowing to your skin to stop it from cracking, peeling and developing ulcers.
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.
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.
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.
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:
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.