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“It Is Not Very Well Known”
Doctor Olaf Schultz; © beta-web
MEDICA.de spoke with Doctor Olaf Schultz, a specialist in internal medicine at the University Hospital of Cologne, what is behind the autoimmune disease, how it is treated and why it is still unknown to so many.
MEDICA.de: Doctor Schultz, almost nobody has heard about the disease called Sjögren’s Syndrome. What is it about?
Olaf Schultz: It is a chronic autoimmune disease that affects the exocrine glands. In this case, the body’s glands are practically destroyed by an inflammatory response of the immune system. This particularly affects the glands around the head and also the lachrymal glands in the eyes or the mucous membranes of the mouth. But it can also manifest itself in the viscera.
Sjögren‘s patients often stand out by the so-called Sicca Syndrome, the dry mouth syndrome. They have dry eyes and the feeling of having sand in their eyes or a dry mouth, causing problems swallowing and chewing. In women it can cause vaginal dryness, so that sexual intercourse is painful.
These symptoms usually lead the patients to see a doctor in the first place. However the symptoms can also, just like with other rheumatic diseases be accompanied by general symptoms like for example faintness, abnormal fatigue or exhaustion. Musculoskeletal discomfort can also occur and joint and muscular pains. In essence it is an illness many physicians might be confronted with, since patients with dry eyes see an ophthalmologist and those with a dry mouth visit an ENT physician or dentist. This is why it is important that many doctors know about this disease pattern.
MEDICA.de: Do physicians generally know about this disease?
Schultz: It is not very well known. In fact, the actual Sicca Syndrome occurs very often, especially in older people, but of course it can have very different causes. Sjögren’s Syndrome is not always the cause. As far as incidence is concerned, it’s said that 0.2 to 0.5 percent of the total population are being affected. At an older age it affects about one percent of seniors.
MEDICA.de: Are men and women equally affected?
Schultz: Women are significantly more affected, about 10 times more often than men – just as with most autoimmune diseases. This is traced back to the female sex hormones, because estrogens influence specific mechanisms of the immune response. This is why it sometimes leads to an exaggerated immune response. Many autoimmune diseases such as rheumatoid arthritis or collagenosis are more prevalent in women than men.
MEDICA.de: Can children also be affected by this disease?
Schultz: That’s very rare. There are two peaks in terms of age of onset. The first one is between the ages of 20 and 40 and the main peak occurs after age 50 and 60, respectively.
MEDICA.de: Can you still hope at a younger age that you get cured from this disease?
Schultz: The course of the disease is extremely variable. There are very mild courses which cause almost no problems, but also very severe courses, where you need to explore all treatment options. Several patients also exhibit increasingly worsening symptoms, which can for instance be accompanied by ulcerations of the eyes, to where eye-sight is strongly impaired. In some cases you need to consider corneal transplants to maintain eye-sight.
MEDICA.de: How is a diagnosis established?
Schultz: For one you have to be sensitized to establish a diagnosis, if a patient for instance exhibits the Sicca Syndrome. Then you need to initiate diagnostics. There are specific tests at the affected organs to objectify dryness. This is to say, to objectify eye dryness, a simple test is run. You place a kind of blotting paper in the eyelid and measure the produced tear fluid. In addition you perform a slit-lamp examination, to check the cornea for defects.
A simple test can detect a defective gland production during reduced saliva production. The patient is asked to chew on a ball of absorbent cotton that is weighed before and after. Depending on saliva production, afterward it has a different weight.
There are of course other examinations, scintigraphy for example or ultrasonic testing, which picture the glands very well and show specific changes of the glands. MRI images also are able to illustrate these soft tissue structures.
The autoimmune disease can also cause sexual problems in the partnership; © panthermedia.net JCB Prod
MEDICA.de: Depression is also said to occur in patients with Sjögren’s Syndrome. How does this happen?
Schultz: In my opinion there is no direct correlation. However, it is actually possible that aside from manifestations on the gland, extraglandular manifestations may occur. That is to say, almost all apparatuses can be affected. For instance, there could be changes in lung parenchyma or interstitial changes of the kidney (nephritis). In the same way, the gastrointestinal tract, the liver or the central nervous system can be affected.
Thus it could lead to nerve damages in the peripheral nerves. But it is disputed whether these changes can be indeed attributed to Sjögren’s Syndrome. It could also for instance be attributed to aseptic meningitis or other causes. The delimitation from Sjögren’s Syndrome is therefore often not easy. At the moment it is still unclear, whether depression is part of Sjögren’s Syndrome or not, since depression of course can also be secondary to these illnesses, because the patient for instance is strongly impaired by these symptoms.
MEDICA.de: How is Sjögren’s Syndrome treated?
Schultz: The first pillar of treatment is symptomatic therapy. For Sicca symptomatology, you for instance prescribe substitute tear fluid to minimize the urge to rub the eyes. You can also try to increase the amount of tear fluid, so the cornea doesn’t dry out, by for example closing off the tear duct. This means that the tear fluid can no longer drain off. You can use candy or chewing gum to combat dry mouth and stimulate saliva secretion. I have one patient who chews gum by the kilo to get relieve. Although there also is an artificial saliva spray, it is not well received by patients, because it is not very pleasant.
If patients also exhibit joint pains, NSAR (non-steroidal antirheumatic agents) are prescribed. On the other hand, immune-suppressant drugs that are prescribed for classic rheumatoid diseases unfortunately are often not effective with Sjögren’s Syndrome. Many drugs were being tested, without any of them proving that they have long term lasting effects. There are newer meds, so-called biologicals, which sometimes attain an effect in severe organ involvement. This includes for example an antibody that is effective against B lymphocytes.
MEDICA.de: Is this area being further researched?
Schultz: At the moment, some of these biologicals are being tested by trying to specifically inhibit those mechanisms that led to the autoimmune process. But we are only beginning to understand this disease. It is far from being as well researched as rheumatoid arthritis for instance. However, there are many research teams that are currently dedicating themselves to this subject.
MEDICA.de: Will patients soon be able to benefit from new therapies?
Schultz: That remains to be seen. You cannot forget that the cancer risk in these patients is strongly elevated due to the permanent stimulation of the B lymphocytes. For example, they are twenty times more at risk to come down with Hodgkin’s lymphoma. And in fact about five percent of patients with Sjögren’s Syndrome also contract such a lymphoid cancer. You need to keep this in the back of your mind for patients with a highly active illness course. Therefore you should regularly check the lymph nodes to prevent lymphoid cancer, because nowadays an early diagnosis leads to a good prognosis.
The Interview was conducted by Simone Ernst
(Translated by Elena O'Meara)
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