Sjögren’s syndrome


Sjögren's syndrome

Sjögren's syndrome
Chronic systemic inflammatory autoimmune disease that can cause death if: lung infection, lymphoma or renal failure.
The T & B lymphocytes of the immune system invade the exocrine glands of your body.
Eventually this invasion leads to ductal hyperplasia of epithelial cells, which cause a duct obstruction.
It subsequently leads to atrophy, fibrosis and acini hyalinization.
Even later, atrophy and replacement with parenchymal fat occurs.
B lymphocytes can cause lymphoid follicles with germinal centers in the salivary glands. 40 times more likely to develop non-Hodgkin's B-cell lymphomas.
Females are more affected than males
Symptoms: Alopecia (dryness), Dry: skin, eyes, nose, mouth, respiratory tract, vagina.
Dry skin can be treated with lubricants.
The dry vagina can cause dyspareunia, which is a painful sexual intercourse. This can also be helped with lubricants.
Dry eyes, also called keratoconjunctivits sicca for more than 3 months is an important sign for Sjögrens syndrome.
The patient often complains of poor vision, photosensitivity, and irritation of the cornea.
Tests: Schirmers test, slit lamp (tear film rupture time)
Treatment for dry eyes: Avoid drugs that cause dryness, for example anticholinergics, diuretics, antihistamines, antidepressants, artificial tears or lubricating drops like methylcellulose or hypromellose.
Stimulate lubrication of the eyes locally with cAMP or cyclosporin 2% of olive solution, or we can systematically stimulate with pilocarpine or cevimeline.
small plugs placed in tear drainage ducts, corneal transplantation.
Dry mouth or also called xerostomia of more than 3 months is also an important sign of Sjögrens syndrome.
This patient is saying she has a dry mouth everyday with difficulty chewing, swallowing and even the taste of food is not as good as before.
The treatment for dry mouth is to drink water daily to help swallow, for example.
We can stimulate the salivary glands locally by sugarless gum, lozenges, or oral rinse containing carboxymethylcellulose.
We can also systematically stimulate the salivary glands with drugs like Pilocarpine or cevimeline.
We can avoid drugs that decrease the secretion of salivary glands, such as anticholinergics, antidepressants or antihistamines.
This patient also has dental caries, which can be prevented with regular dental visits with topical application of fluoride, or simply maintaining good oral hygiene after each meal.
She is complaining about Candida secondary infection.
In this case topical nystatin, lozenges or clotrimazole are administered.
The stones may appear in the salivary ducts, which must be removed to preserve the salivary tissue.
We can measure the production of saliva, which will be low in Sjögrens syndrome, ie less than 1.5 ml in 15 min.
We can also use techniques such as saline scintiscanning or sialography.
Unless dry, the patient may have enlarged parotid glands
There are also extraglandular symptoms.
So the most common is arthralgias and arthritis. We treat it with hydroxychloroquine or methotrexate and prednisolone.
Raynauds phenomenon can be seen, and its treatment with nifedipine and gloves that protect from cold.
Lymphadenopathy can be seen in the cervical or axillary region.
Vasculitis may present as rashes called purpura, which can be treated with glucocorticoids or immunosuppressive agents such as cyclophosphamide.
Kidney diseases such as interstitial nephritis, kidney stones, impaired ability to concentrate or renal tubular acidosis. A bicarbonate replacement may be given for renal tubular acidosis.
Chronic Hepatobiliary Diseases
The risks of non-Hodgkin's lymphomas may be 40 times higher in patients with Sjögrens syndrome.
This can be treated with anti-CD20 therapy along with a CHOP regimen, which is a regimen of chemotherapy consisting of Cyclophosphamide, Hydroxydaunorubicin, Oncovin (Vincristine), Prednisolone.
Splenomegaly or myositis can rarely be observed.
If we do blood tests, we can see leucopenia, anemia, a higher sedimentation rate of Erythrocyte, and some autoantibodies.
Autoantibodies are rheumatoid factors and antinuclear antibodies called SS-A (Ro) and SS-B (La).
Except for blood tests, we can confirm that it is Sjögrens syndrome by taking a biopsy of the lip, more specifically of the minor salivary glands in the buccal mucosa.
This will demonstrate in the microscope that the lymphocytes have attacked the salivary glands
There will be many large areas of lymphocytes with tissue atrophy.
Genetic testing may show an association of Sjögrens syndrome with HLA DR3.

Primary
Secondary
Rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, mixed connective tissue disease, vasculitis, Hashimoto's thyroiditis, polymyositis, primary biliary cirrhosis and chronic autoimmune hepatitis.

Video credits to Dr. Andras Fazakas YouTube channel





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    Sjögren’s syndrome

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