THE PROBLEMS OF SJOGREN'S SYNDROME
by Doctor C. W Hutton, Consultant Rheumatologist, Derriford Hospital, Plymouth
Sjögren's Syndrome causes many problems for patients. There is a continuing difficulty in understanding its aetiology, working out the diagnosis and planning out the most appropriate management. However, progress has been made. Many of the advances in other areas of medicine are having direct effects on the clinical problems which patients with Sjögren's have. Advances in understanding other diseases, particularly in relation to the way in which the immune system is working are likely to have very helpful insights into understanding Sjogren's syndrome in the future.
The main clue as to the aetiology of Sjogren's syndrome is the role of the immune system.
We know that Sjogren's is associated with a lot of other conditions which appear to be due to malfunctioning of the immune system, for example in rheumatoid arthritis or in systemic lupus erythrematosus.
Clinically, there are features of immunological abnormality, both in testing the blood and looking at the biopsy of tissue from patients with Sjogren's syndrome. The difficulty is in understanding what triggers it and what then causes the problem to progress, why does it relapse and remit, with a small number of patients getting a very severe problem affecting many tissues in the body.The prime tissue "target" though is glands which produce fluid - the exocrine glands. The most important are around the gastrointestinal tract, but the dryness problem will affect the production of tear-film, the production of saliva, the correct hydration of the nose, the normal lubrication of tissue of the lining of the vagina, as examples. It is important to understand that none of the components of an exocrine gland is simple water. They are complex combinations of material designed for a particular task. Tear-film is designed to lubricate the eye. It needs to stick onto the eyeball and not to flow away from the eye. It needs to contain material to prevent bacteria from infecting the eye and acts as a cleaning mechanism, removing dust. Consequently there is not only a problem with the amount of fluid produced, but also the character of the fluid.The diagnosis of Sjogren's syndrome is based on a clinical assessment. This is then supported by the identification of specific abnormalities. These may be found, looking immunologically.
There may be the presence of an auto antibody and in Sjogren's it is helpful to identify whether a patient has an anti nuclear antibody and antibodies which have a fairly high specificity for Sjogren's, which includes antibodies to sub-mucosal Ro and La. It may also be helpful to have imaging of the salivary glands with technetium isotope scans, which show diminished activity of the salivary glands, which fail to respond appropriately to stimulus.Definite confirmation of diagnosis is by biopsy, best done by biopsying the little mucosal glands in the mouth. The problem though is that this is much easier when someone has more advanced, severe disease than early change. It is then much more difficult to interpret the information early in the process and it is at that time that many patients are very frustrated, as they seek an ubderstanding of why they feel so dry.
It is important to appreciate that a number of other conditions can mimic the pattern of the dryness of Sjogren's syndrome, particularly in the early phase. A combination of fatigue, a feeling of dryness and widespread aching may, for example, present as part of depression or fibromyalgia and may indicate the development of hypothyroidism and not indicate that someone is going to get this progressive condition.The management of Sjogren's syndrome remains primarily about the management of the symptoms. Three main areas of symptom control are reducing dryness, improving pain control and controlling fatigue. There are a number of preparations which really need to be reduced on a trial and success basis, rather than a trial and error basis, to find out what the most appropriate treatment is for an individual, for example in terms of artificial tears and artificial saliva. Pain control needs an understanding of why patients get pain, if it is due to an arthritis causing a synovitis, then anti inflammatory medication may be helpful. If it is less precise, then analgesics like regular paracetamol may be appropriate. The important thing, however, is for patients to under-stand that this is not something which can simply be abolished. It is a question of trying to make it more tolerable.A similar issue surrounds the improvement of the problem of fatigue. Sjogren's syndrome appears to directly affect the mechanism of well-being and that may well be subjectively experienced as fatigue. It is important to distinguish between peripheral fatigue, associated with abnormality of the muscle, which can occasionally be due to inflammation of the muscle, and central fatigue associated with weariness. The latter is much more common and is actually much more difficult to treat. However, careful attention to factors which are controllable is the mainstay. If people can focus on making sure that they get appropriate and restful sleep, then they go a long way to breaking a cycle of continually feeling worn out. Secondly many patients experience worsening fatigue if they do increased activity, so tailoring the amount of activity to what they expect they can tolerate, is critical to keeping the fatigue to a tolerable level.In many respects now, it is an exciting time for progress in understanding disease. There is phenomenal advance going on in biology, which is going to feed into understanding conditions like Sjögren's syndrome. The second major change in the information revolution is much more availability of excellent information about conditions like Sjogren's. Web sites of both the British and American Sjogren's Syndrome Associations are extremely helpful. However, patients who have a particular condition and a particular need for that, need to appreciate that it is no longer possible for many doctors to maintain the level of expertise in knowledge that was possible when the world was a simpler place. A doctor is much more someone to share information with, to get a perspective on the problems, rather than someone who takes a role which is more directive
Sjogren's Syndrome : An Advisory Guide for Patient's and Doctors : £2.50
This booklet is produced by BSSA and is an invaluable source of information on Sjogren's syndrome both to newly diagnosed patients and also G.P.s, junior hospital staff, dental surgeons and nurses who will find the contents helpful in understanding the problems of paitients with the disorder.
Note:
WebHealth has additional articles about Sjogren's Syndrme at:
Systemic Features By:
Dr Ian Griffiths, Dr Elizabeth Price, Dr Clive Kelly and Dr Patrick Venables
and
The Problems of Sjogren's Syndrome By:
Dr CW Hutton, Consultant Rheumatologist, Demford Hospital, Plymouth
and
Sjogren's Syndrome and the Gastro-Intestinal Tract By
Doctor Richard Mount Ford MD, FRCP, FRCR.
Consultant Physician, Department of Gastroenterology, Bristol Royal Infirmary
and
Neurological problems in Sjogren's Syndrome by Doctor Annabel Coote and Doctor Michael Snaith, Department of Rheumatology, Royal Hallamshire Hospital, Sheffield.
and
Sjogren's Syndrome and the Respiratory System
A Brief History of Sjogren's Syndrome By
Doctor Ian D Griffiths FRCR, Consultant Rheumatologist,
Freeman Hospital, Newcastle-Upon-Tyne
