Chris Denton is a Professor in the Centre for Rheumatology at the Royal Free Hospital in London and runs the Scleroderma Clinic. He also has a major research programme that spans both clinical and laboratory trials related to scleroderma. He is a medical advisor to the RSA and very grateful for the questions that he is challenged with in our newsletters (Hot News).
Do you inevitably develop scleroderma if you have severe Raynaud’s?
The answer is very definitely no. There have been a number of studies which have been undertaken where people have followed up the severity of Raynaud’s in terms of things like the frequency of attacks etc. What does appear to put people at risk are firstly some of the nail capillary changes and also if people have abnormal antibodies. They will have some abnormal proteins in the blood which can sometimes be present for some years and do seem to predict what is going to happen. The key thing is that Raynaud’s can be mild or it can be severe but even amongst those with severe Raynaud’s in terms of the number of attacks only a very small minority will go on to develop scleroderma.
I have CREST and over the past few weeks I have experienced a strange sensation over my chin going towards my cheek. It feels numb for a short time. Is this anything to be concerned about?
This is an unusual symptom, but one possible link with lcSSc (CREST) is that the nerves sometimes become involved in scleroderma causing numbness or altered sensation in areas of the face or in the mouth or tongue. Sometimes this is painful (neuralgia) in other cases there is numbness. This symptom should be discussed with your doctor. For these problems some medications that reduce nerve irritability can be effective such as gabapentin or pregabalin amongst others.
I have Raynaud’s and my hands swell up – am I developing scleroderma?
Most people with Raynaud’s do not have scleroderma - and swelling of the hands can be normal in Raynaud’s that occurs in otherwise healthy people (primary Raynaud’s). However you should discuss this with your doctor as you may require tests to look at the blood vessels around the finger (nailfold capillaroscopy) and blood tests for autoantibodies. If these tests are normal you are very unlikely to have scleroderma.
I have the systemic form of scleroderma and am breathless but my doctor says all the tests are normal - should I worry?
Breathlessness is common in scleroderma and can reflect serious problems in the heart or lungs, or a low blood count (anaemia). However, if heart and lung tests are satisfactory it may simply result from deconditioning of the muscles. If your symptoms are stable you should have echocardiography and lung function tests performed at least once a year to make sure no serious problems have arisen.
My doctor has diagnosed me with Raynaud's. Do I need to see a consultant and if so what kind of consultant?
It is important to clarify whether this is primary or secondary Raynaud’s, the latter associated with underlying diseases such as scleroderma. Most often a rheumatologist is the best person to see as most of the associated diseases are rheumatic, such as lupus or scleroderma. Sometimes vascular surgeons or dermatologists may have a special interest in Raynaud’s.
I was diagnosed with scleroderma in 1991 and have many symptoms including Raynaud's and gut problems. I have been having dilatations of the oesophagus regularly for many years but last January I had a dissolvable stent fitted where the stricture is. It has changed my life and enabled me to go out to eat and to eat most things. I also take Domperidone. What are the most effective medications for Acid Reflux?
Almost all patients with scleroderma suffer from heartburn and reflux symptoms. The inflammation of the oeophagus may result in scarring or a stricture. This is much less common that in the past as drugs that suppress acid, such as omeprazole, are very effective. Dilatation and related procedures as you describe can be very effective but must be followed by full treatment to suppress acid (such as omeprazole) and improve motility (e.g. domperidone) to reduce the likelihood of recurrence. The most consistently effective drugs are those in a class called ‘proton pump inhibitors’. These reduce stomach acid and help reflux but higher doses than normal may be required.
I have recently filled in a questionnaire for the RSA and the first question was “Do you have diffuse or limited scleroderma?”. I have never been told which I have so how can I find out? Please can you explain the difference in simple language?
Scleroderma is the medical term for “hard skin” but is often used as a shorthand for systemic sclerosis, a condition which is characterised by thickening or hardening of the skin that varies in extent, and also by abnormal circulations (e.g. Raynaud’s phenomenon). In some cases damage to internal organs occurs such as in the heart, lungs, kidneys or bowel. Traditionally systemic sclerosis is divided into two main types (or subsets), limited or diffuse. In the diffuse form there is skin thickening over the upper arms or legs or chest and abdomen. In the limited subset only the skin of the face or extremities are affected. Usually the subset becomes apparent within the first 2 or 3 years of the disease and patients do not move between subsets. The diffuse form and limited form have different patterns of internal complications and so the subset definitions are helpful to specialists in managing the disease. Your specialist should be able to tell you which form you have, aided by the results of specific blood test markers such as anti-nuclear antibodies.
How will I know if I have scleroderma? I have looked at a list of symptoms and have four of the five listed. I was diagnosed with Raynaud's last year after my hands started to ulcerate. My hospital visit to see the rheumatologist lasted all of two minutes and I never received a follow-up appointment.
Diagnosis of scleroderma requires expert assessment including examination of the skin, blood tests and sometimes additional investigations. Almost all patients with scleroderma have Raynaud’s but there are also many other causes of Raynaud’s phenomenon and so you should clarify what tests have been done and seek a further expert opinion from a rheumatologist or other specialist if your GP is concerned.
I have recently been diagnosed with scleroderma and have had very itchy skin. Is this likely to improve or will it always itch like this? Is there anything you can recommend to help me?
Itching of the skin can occur for a number of reasons in scleroderma – it is particularly common in the early active stages of diffuse subset of scleroderma and can be very problematic. Anti-histamines and other treatments may help and tests for other medical causes of itching should be performed (e.g. iron levels and thyroid function tests). In general itching improves as the skin disease stabilises but this can take some time. Itching is less common in later stages.
I have read that early diagnosis of scleroderma is important. Does this mean that if I get treatment early it will make my symptoms less or just stabilise the symptoms I already have?
It is important to diagnose scleroderma so that any complications can be addressed. One of most important aspects of the disease is to screen for any lung or other internal organ complications and this can only start once a diagnosis is established. We believe that early treatment improves overall disease outcome.
