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Frequently Asked Questions

A: Bloating and uncomfortable feelings of abdominal fullness and pain after eating are common in scleroderma (Systemic Sclerosis). Reasons include slowing of emptying of the stomach and also a tendency for bacteria to overgrow and cause extreme wind in the bowel.

Lamoprazole is very effective in treating heartburn in scleroderma by switching off stomach acid. Unfortunately this removes the natural anti bacterial effect of the acid. Antibiotics can kill the bacteria. You should discuss with your doctor whether this is something that might help you. A specific breath test can help confirm bacterial overgrowth.

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Q: I have scleroderma and Sjorgens Syndrome. Which is more likely to have caused central nervous system problems?

A: Nervous System changes occur in connective tissue disorders, but more often in Sjorgrens Syndrome than Systemic Sclerosis. The common effects are problems with the peripheral nerves (neuropathy). However there are reports of neurological complications in scleroderma too.

Systemic Sclerosis is very variable but can cause scarring of many organs including skin, lungs, heart, bowel and kidney. Fortunately the majority of patients do not have all of these problems.

A: There is no theoretical reason why either shaving or waxing should be a problem. Waxing might have an additional therapeutic benefit, but removal using the wax method may have the potential to be more traumatic to the subcutaneous tissue. On balance shaving is probably less traumatic. So, a slight preference for shaving.

A. Scleroderma is multifactorial there are a lot of thing's that go together to cause the disease, there's some evidence that some of those things are genetic in the sense that they are part of your genetic makeup which obviously you share with your relatives. What we do notice and what has been shown in families is that it's very very unusual for scleroderma to occur more than once in a family. Sometimes other features that are seen in the disease can occur so patients can have abnormal blood tests or perhaps another disease that may be a little related like lupus or they may have Raynaud's phenomenon. Quite often you see in a family a number of patients with Raynaud's but only one with scleroderma. In fact we have looked at families with Raynaud's. If you have a family with Raynaud's and you do have scleroderma it seems actually statistically even less likely that your relatives will progress. So I think all that we can say is that there are common genetic factors and perhaps on occasion, even though patients are separated geographically, if they've got enough similar genetic factors maybe if they are also coincidentally exposed to some other trigger both may develop the condition.

A. Care of your teeth is very important there are some problems associated with the condition and if this becomes problematic either discuss them with your GP or Rheumatologist, who will be able to refer you to someone with more experience in this field. Peter Ndenga offered the following advice: tell your dentist before the start of treatment of any new symptoms and discuss medication, for example if you are on Warfarin you will take longer to stop bleeding. This is useful knowledge for your dentist to know before he begins treatment.

A. Treatment of fingertip ulcers over areas of calcinosis is very difficult. Sometimes these ulcers can be infected, when an antibiotic is required. Unfortunately there is currently no effective treatment for the calcinosis itself. If the calcinosis is a big problem, then surgical treatment may be indicated, although surgery to the fingers in-patients with scleroderma does carry risks, because of poor blood supply and skin thickening. Even surgery is not curative - it simply reduces the 'bulk' of the calcinosis. Some surgeons will 'drill' the calcinosis out with a dental drill.

A. There is a good theoretical basis for using CGRP in patients with severe Raynauds and where the levels of natural CGRP are low in the blood vessels - based on research studies. However there are no major trials. CGRP is used for patients who find Iloprost hard to tolerate at effective doses or due to side effects, or when it is ineffective. Both treatments are given by intravenous infusion. Iloprost has been shown to be very helpful in Raynaud's and is probably more powerful than CGRP and is our first choice drug.

A. These spots are called telangiectasia. They are dilated superficial blood vessels seen on the face and hands of scleroderma patients. They constitute a cosmetic problem to the patients; corrective make-up can be used with benefit. Telangiectasia are often resistant to medical treatment. Dermatologists nowadays use new technologies such as laser therapy, where a high energy light is used to obliterate dilated blood vessels. This treatment can be painful, only few telangiectasia can be treated at one time and it may leave some bruising for a few days. Unfortunately, recurrence after treatment is common.

A. The control of the blood flow is in part dependent on the sympathetic nerves, which can constrict blood vessels. Vasospasm is a feature of Raynaud's phenomenon and can be sometimes improved by "destroying" the sympathetic nerve supply called sympathectomy. Sympathectomy is a surgical procedure performed under a local anaesthetic. It can be digital, i.e. applied to the individual finger(s) and useful for ulcers on the fingers; cervical i.e. applied to the upper limb, for Raynaud¹s in the hands or lumbar i.e. applied to the lower limbs, for Raynaud¹s affecting the toes and feet. Benefit is often variable and temporary, and therefore this procedure is now rarely used. Side effects may include swelling and burning sensation in the extremities, dry skin and scarring.

A: Tiredness can be a symptom of connective tissue diseases such as scleroderma. However, it is important to check out that there is not another reason, such as anaemia, or an underactive thyroid, and so your doctor may want to arrange some extra checks, especially if this is a new problem'.

A: Firstly you need to be assessed by a gastroenterologist which seems to be in hand. Yes gluten sensitivity occurs in some scleroderma patients, an antibody test and biopsy of the Jejunum is required.

A: I am not aware of any relation between scleroderma and alcohol. There are one or two short papers where the authors report a higher alcohol consumption (as well as smoking or dental filling) in patients with Systemic Sclerosis. But there is obviously no causative effect documented so far. Moderation is always good, but not essential. Alcohol can make Oesophagitis worse. The only thing one should watch carefully is what sort of treatment the patient is having: in particular NSAIDs, Methotrexate or Azathioprine. In this case one should cut down or stop drinking.

A: Scleroderma in its systemic form (systemic sclerosis) divides into two patterns. Patients with diffuse scleroderma have tightness of skin over upper arms, thighs, chest and abdominal wall. In the limited form of the disease (once called CREST) there is tightness only on the extremities. You should retain the subset classification that you have in the first year or two of disease. However in some patients with diffuse scleroderma there is a major improvement over time, especially after three years, and the pattern of skin involvement may eventually resemble limited scleroderma. Hence the confusion. Really a doctor should ask if the body was ever affected when they first try to classify you.

A: As in other areas of medicine and biology, we have leant a lot from other species. In particular there is a naturally occurring mouse that develops a condition similar to scleroderma. This mouse has been studied and has given important clues about the way in which a protein called fibrillin, that regulates the elasticity of skin and blood vessels, might be altered in scleroderma.

A: Foot pain can arise in several ways in scleroderma. Early in the disease there can be arthritic pain from joint inflammation. Later Raynaud's attacks can be painful especially if they lead to ulcers over the toes. Raynaud's can also lead to burning discomfort when the feet warm up after an attack. However the commonest cause of foot pain is later in the disease, often after several years. There is shortening of the tendons and loss of soft tissue within the feet that leads to mechanical foot pain. This can be helped by a podiatrist, who may advise orthoses (e.g. insoles). Foot-wear must be appropriate - good arch support, well fitting and warm.

Another less common cause for foot pain is an irritability of the nerves. This is called a neuropathy or neuralgia and can be associated with numbness as well as pain. Typically the pain is burning in nature and treatment with drugs to reduce nerve irritability may be required.

A: Unlike in lupus (SLE) there is no direct evidence that UV light is harmful in diffuse scleroderma. In fact some studies have even investigated whether phototherapy using UV light actually helps. However, an exposure to the sunlight does aggravate the pigmented changes in scleroderma, perhaps by activating pigment producing cell.

Q:- How safe are prescribed steroids?

A:- Steroids are a very useful tool for use in rheumatological situations. However like all drugs they have side effects. The aim of your doctor is to use them to their maximum benefit and if long term treatment is required to either replace them with another 'disease modifying agent' and/or to keep the steroid dose low to minimize their long term side effects. We are well aware of their potential to cause osteoporosis for instance and your doctor will often prescribe bone protection at the same time.

Q:- I have been advised to take 400mgms of Vitamin E daily and have recently read articles about this dose being dangerous for elderly people. Some have died by taking this dose. I am in my late 70's and want to know how safe this dose is.

A:- The studies you recall involved a very small group of patients and many had coexistent diseases to account for the increased mortality rate. We recommend about 200-400mgms daily.

Q:- What supplements do you recommend people with Raynaud's and Scleroderma to take?

A:- Vitamins A and E are classified as antioxidants and are thus beneficial to the circulation. We would also recommend evening primrose oil, fish oil and some patients find Ginko Biloba very helpful.

Q: Is there medication that can manage pain and stiffness in the hands effectively?

A: Pain and stiffness in the hands is a common problem in scleroderma.

This may be due to a number of problems and each would have to be assessed by your rheumatologist.

They include osteoarthritis, inflammatory arthritis, calcinosis and finally Raynaud's phenomenon. Some patients may have just Raynaud's but others may have a combination of these and each will require quite different medication or combination of treatments. Overall we would recommend diagnosis in the first instance. We often favour wax treatment, as this is useful for osteoarthritis and for the treatment of calcinosis.

Important Note : When using wax treatment on your hands any broken skin must be protected with plastic gloves.

Q: What is the current medical opinion with regard to the treatment of calcinosis in cases of scleroderma. In particular what are the risks and benefits of surgical intervention?

A: Treatment is difficult but there are a few anecdotal suggestions such as low dose warfarin, probenecid and colchicine which are used in gout, calcium channel blockers such as diltiazem, intralesional corticosteroids, carbon dioxide lasers and anti-TNF therapies used in rheumatoid arthritis.

Recently reported in the Annals of Rheumatic Disease were 9 patients treated with Minocycline an antibiotic. Minocycline was shown to be beneficial to the removal of calcinosis as well as its other properties. This was given in cyclical doses. But the side effects limited its usage.

A well tried method particularly for many patients with calcinosis of the fingers is waxing (as mentioned above). This softens the skin and allows natural extrusion of the calcium. One must always resist the temptation to 'pick' at these lesions as this leads to skin infection and then the need to proceed further with oral antibiotics.

Surgical techniques that are well tried include removal with the aid of a dental drill, but this would require an anaesthetic and does not guarantee permanent removal. A few patients require surgical removal by plastic surgeons depending on the size, location and degree of the problem.

Q. What is the similarity between gout and scleroderma?

A. They are of course very different diseases but they both have the tendency in a few patients to precipitate calcific deposits in the skin. About one fifth of patients with scleroderma will have calcification overlying pressure areas in the skin which are often painful and difficult to eradicate. In gout some patients with long standing disease will deposit urate in the skin over joints and again pressure areas. These may be removed by appropriate treatment of their gout.

Q. I have localized scleroderma and have had a cataract operation. I now have a film appearing over the same eye and wonder if it's connected with scleroderma?

A. The first thing to emphasise here is that localized scleroderma does not affect other organs other than skin and underlying muscles and occasionally growing bones in children. If you have noticed any new symptoms following your recent surgery you should consult your doctor who will refer you either to an ophthalmologist or back to the surgeon who operated on you. It may be something very simple like dry eyes which is common and often presents with the symptoms you describe but eyes are special and you should always seek expert advice when it comes to eye symptoms.

Q. Are yellow eyeballs a sign of scleroderma?

A. Yellow eyeballs are usually a sign of advanced jaundice if they are truly yellow. Some people have a suggestion of a yellow tint to the eye but may not be jaundiced. There is an association between some types of liver disease and scleroderma. You would be well advised to talk to your doctor who can confirm whether your symptoms are truly significant / pathological. He may well use a blood test to confirm this.

Q. Does scleroderma cause the ageing process?

A. No we are all undergoing the ageing process. If you mean by ageing the normal aches and pains associated with getting older we know that there is an increased incidence of musculo-skeletal problems associated with systemic sclerosis and again you would be advised to seek medical opinion in case the symptoms you are experiencing are due to another medical problem which requires diagnosis and appropriate treatment.

Q. Does scleroderma take the back seat when some other disease comes along?

A. Scleroderma is a chronic illness with some symptoms that are often persistent, but others declare themselves later on depending on the organ involved. Within this framework symptoms often fluctuate with time even with a chronic illness. If one is unfortunate to develop another unrelated illness this may take priority as far as the symptoms felt at that time but usually will not affect the natural progress of a disease like scleroderma. We would never take the issue of scleroderma as a back seat disease!

Q . I have just found out that my ten year old daughter has Morphea Scleroderma. It started out as a quarter size white lesion on her leg and within the last three years it has spread down to her knee and up to her private parts. We live in the States and I just wanted to know how other countries feel about Methotrexate and Steroids? Western Medicine wants to give her an IV three times a month for three months. I'm really scared because I'm an Eastern Medicine believer. Any help you could give me I would surely appreciate.

A . The first thing to say is that every case is different and has to be individually assessed. Because localised scleroderma or morphoea is an uncommon disease there are no randomised controlled trials in the literature.
Most centres in the West, and particularly in North America and at Great Ormond Street in England, would advocate intravenous methylprednisolone and methotrexate as the standard treatment for this condition and there are many anecdotal reports to support this. If there are signs of progression of a lesion on the skin, and the best way of assessing this is usually relying on the patient's description or parent's observation. We would recommend active treatment in this way, increasing the dose of methotrexate until control of the disease is reached or until the patient becomes intolerant of methotrexate. Often conversion from oral to subcutaneous injections helps with the troublesome gastrointestinal side effects. We have also had success with oral cyclosporin, oral or topical mycophenolate mofetil and topical tacrolimus ointment as alternatives.

Q. My Father had a very aggressive form of systemic sclerosis and I recently had an ENA Blood test which proved positive for lupus antibodies. I have since had further blood and organ tests which are negative. Could you comment please?

A. It is common to have a connective tissue antibody in a close relative of a patient with scleroderma or any of the other connective tissue conditions. It reflects "background" susceptibility but is extremely unlikely to develop into disease as multiple other triggers are needed.

Q . What is the likely outcome and expected survival with systemic sclerosis?

A . This largely depends on which organs are involved. In the past patients died more often from renal complications but we are much better able to treat patients with this complication nowadays. Renal crisis occurs more often in patients with diffuse cutaneous systemic sclerosis in the first two years after presentation, so by educating patients to report the early warning signs we can save their kidneys by initiating early treatment.

Pulmonary hypertension which occurs in isolation in some patients with limited cutaneous systemic sclerosis carries a poor prognosis, but again we have much better treatments for this and by monitoring our patients with yearly lung function and echocardiogram we are able to treat them early.

For the vast majority of patients the outlook is good. So that we can usually be reassuring and treat them for their circulation problems and bowel symptoms with well tried treatments.

Q. Are plaques in Morphoea a sign of inflammation and increased blood flow? Is there a difference between new and old plaques?

A. Pain is due to a combination of factors. There is often loss of connective tissue or the soft padding on the sole of the feet which tends to cushion the pressure between the bones and the floor. So one feels as though one is walking on pebbles and this can be quite painful with the development of hard callous on the sole. We would recommend wearing insoles to the shoes.
Raynauds also contributes to the pain particularly in cold weather and this can be treated with a variety of medications or a lumbar sympathectomy. We are always mindful of the role of the large vessels contributing to the circulation in the feet, so we sometimes arrange to do vascular studies to see if there is an obstruction to these arteries which might be amenable to surgery and thus improve the circulation to the legs and feet as a whole.

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Q. What is a lumbar sympathectomy?

A. Normally small arteries or arterioles are kept partially shut by the sympathetic nerves. If the tissues need more blood the nerves become less active and the blood vessels widen in order to let more blood through. The sympathetic nerves travel down the back adjacent to the spinal column. These can be disrupted by an injection of phenol, under local anaesthetic, to improve the symptoms of Raynauds of the feet. This is usually preceded by a temporary injection on one side in order for the patient to experience the benefit before consenting to the permanent injection. Generally some relief is felt immediately but it may take some time before maximum benefit is felt. Side effects include increased dryness of the skin, occasional burning sensation of the extremities and swelling of the legs.

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