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By Dr. M. Walravens, rheumatologist, O.L.Vrouw-Clinic Aalst
|What is Sjogren's Syndrome?|
|Sjogren's Syndrome (SS) is a disease in which the lacrimal and salivary glands are chronically inflamed. This causes deficient tear and saliva production with possible complaints of dry eyes and dry mouth. When it occurs by itself it is called primary SS. If it occurs as a part of another autoimmune disease it is called secondary SS. SS can be considered a point where all the autoimmune diseases meet. Which means that a lot of autoimmune diseases have SS in common. In SS overactive immunological cells (lymphocytes) may turn out to be malignant. Fortunately this is rarely the case. This is why SS is considered an important link between autoimmunity and malignancy. |
|SS is not a rare disease. It follows rheumatoid arthritis in number, which is a very common disease. Ninety percent of the patients are females and although it can occur at all ages, the onset of the disease is usually situated round the menopause (at 43 years on average). Familial types also occur. |
|Most important symptoms|
|In primary SS first complaints are usually dry eyes or dry mouth, which can be very disturbing. Dry eyes feel sandy or gritty. In the morning, the eyelids may stick together or the eye sight may be blurry due to a film of dried out tears on the eye. Occasionally small wounds caused by insufficient tear production occur on the eye. A dry mouth makes speaking and eating very difficult. Patients need to drink a lot to be able to swallow food. They often complain about pain in the mouth. Caries form more easily and dental prostheses are hardly tolerated, do not stay on and sometimes cause pain. Swollen salivary glands, and especially swollen parotid glands occur occasionally. Only rarely is the swelling very extreme. |
|Secondary SS will often be discovered after other diseases like lupus or rheumatoid arthritis have been diagnosed. Laboratory test results may be abnormal without the patient experiencing complaints. Increased production of antibodies, the production of autoantibodies (especially anti-La and anti-Ro), a low amount of white blood corpuscles and the presence of the rheumatoid factor are typical. |
|Dry eyes and/or mouth together with one or more significant abnormality in lab tests are sufficient to make a diagnosis. |
In case of doubt, a lip biopsy, by means of which small salivary glands are examined, may be helpful to determine the diagnosis of Sjogren's Syndrome. Since practically all biopsies show abnormalities, however, it is not a reliable test to diagnose Sjogren's Syndrome. A lip biopsy heals slowly and may leave a scar. It may also be painful.
In a sialography contrast-fluid is injected in the salivary duct. This is quite a difficult and disagreeable test and it is rarely used.
A scintigraphy is less complicated and is more comfortable for the patient. A radioactive substance is injected into the blood. It accumulates in the salivary glands and is secreted together with the saliva. In this way, the functioning of the salivary glands can be measured. The ophthalmologist can measure the production of tears by means of the Schirmer test (a slip of paper absorbs the tears) or by means of colouring agents.
|Involvement of other organs in SS|
|I In secondary SS all symptoms of the accompanying connective tissue disease may be present.|
Organ involvement is also often found in primary SS:
- Dryness of the bronchial tubes causing a tickling cough
- An invasion of lymphocytes (immunologically active cells) may inflame the connective tissue in the lungs. Foci (points of lodgement and development) of these cells may provoke a dry cough or shortness of breath
- A dry mouth or throat may cause swallowing difficulties, but are rarely provoked by involvement of the upper part of the oesophagus
- Reduced secretion of gastric juice occurs but is seldom the source of complaints, although it may evolve into a type of anaemia
- Secretion of the pancreas may be reduced, mostly without observable consequences
- The liver may show signs of autoimmune involvement; primary biliary cirrhosis or chronic hepatitis
- Kidney involvement usually causes insufficient urine concentration (with nocturia, i.e. urinating at night, as a result) or acidosis, a slight souring of the blood
- Purpura (subcutaneous bleedings) are rare
- Joint pain, mostly with swelling, occurs in two out of three patients
- Involvement of the central nervous system may occur with neuralgia in the limbs, and rarely causes severe damage to the spinal cord or brain. Psychiatric complaints are common: mostly depression, but also concentration problems, difficulties to memorise, personality changes and sleep disorders
- Allergies to medication are very common in SS
- Hyperviscosity seldom occurs. This happens when the blood becomes thicker due to too many antibodies (sometimes also rheumatoid factor) in the blood. Symptoms may vary: mostly headaches, blurry vision, confusion, nose bleeds and bruises. It is a serious problem that requires immediate treatment with plasmapheresis followed by corticosteroid treatment.
- Extreme and sometimes sudden fatigue
|Autoantibodies are directed against cell components and more specifically against the nuclei of cells. That is why they are called antinuclear (nucleus = centre) antibodies (ANA) or antinuclear factors (ANF).|
The ANA test is performed in a lab by adding a fluorescent dye to the blood serum. In this way, antinuclear antibodies can be detected under a microscope. Antinuclear antibodies are present in 40 % of the patients.
Further examination shows primarily a pattern of antibodies against one specific group of cytoplasmatic antigens, especially against La/SSB and Ro/SSA. More specialised techniques could enable the detection of these antibodies in a larger number of patients.
|Treatment By Dr I Khan|
|Dry eyes are best treated with artificial tears.|
A dry mouth is easy to treat just use Vitamin B-12 sublingual for complete recovery. Use Omega-3 daily and use Vitamin D daily please see the diet section.
Sucking sour candies and sugar-free chewing gum may give some relief. Drinking lots of water is not recommendable because it flushes away the little saliva and water cannot serve as a substitute for saliva. Sulfarlem or Bisolvon are worth trying. A humidifier may prove useful.
In severe cases low to moderate doses of corticosteroids may help, but high doses are seldom necessary. \
Vitamin B-12 taken by injection or under the tongue is very effective, orally swallowed pills usually do not help.
|SS is not hereditary. The disease, however, is believed to possess an inherited susceptibility. There is connection between the HLA DR3 type and the DRw52 type. Although the exact role in the development of SS is still unknown, these HLA antigens (HLA stands for Human Leukocyte Antigen) are believed to be important for the production of autoantibodies. |