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Sjgren’s syndrome

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Sjögren’s syndrome

Imagine yourself not being able to cry this woman have these problems she have a chronic systemic inflammatory autoimmune disease that can cause death if she gets pulmonary infection a lymphoma or just renal failure so we said that this is an autoimmune disease and in sugar syndrome this means that the t and b lymphocytes of the immune system invades the exocrine

Glands in your body where can we find this exocrine glands lacrimal glands for example secret tears salivary glands like the parotid submar demolar and sublingual glands secrete saliva so there are exocrine glands in many more places like in the skin causing sweating in the nose respiratory tract vagina gastrointestinal tract and many many more so eventually

This invasion leads to ductal epithelial cell hyperplasia which cause a duct obstruction later it leads to atrophy fibrosis and helenization of aciny so still later there’s atrophy and replacement with fat of the parenchyma we can explain it with one word destruction so we mentioned that b lymphocytes are also infiltrating this glands and these b lymphocytes can

Cause lymphoid follicles with germinal centers in the salivary glands the lymphocytic infiltrate can be so intense that it looks like a lymphoma in fact these patients are about 40 times more likely to develop b cell non-hodgkin lymphomas so in conclusion we know that there is exocrine gland destruction in shogun syndrome so this picture now shows the most common

Symptoms and organs affected in this disease here we see a middle-aged woman because females are affected more than maize and usually these females are what middle-aged we can see alopecia which means hair loss we can see dry skin dry eyes dry nose that can cause diminished smell dry mouth dry respiratory tract that can cause cough and dry vagina so the dry skin

Can be treated with some lubricants the dry vagina can cause dyspareunia which is a painful sexual intercourse this can also be held to help with some lubricants the dry eyes also called keratoconjunctivitis sikka for more than three months is an important sign for children syndrome the patient is usually complaining about bad vision photosensitivity and irritation

Of the cornea so which exocrine glands are destroyed in the dry ice it’s the lacrimal glands how do i know that the drives are because of sjogren’s syndrome i mean is there any other disease that can cause dry eyes and yes for example there can be inflammation because of stephen johnson syndrome the panthecoi chronic conjunctivitis chronic blepharitis so there are

Many types of diseases and it can be a simple trauma to die or a blink problem it can also be caused by hypovitaminosis a which is a deficiency in vitamin a there can be neurologic problems so that the eyelids or lacrimal glands are not functioning as they should even drugs can cause dry eyes example the anticholinergic diuretics antihistamines or antidepressants

So as you understand we have to do more tests to make a differential diagnosis the symptoms by themselves are not enough to say that this is children syndrome we can do the shear mass test we put a filter paper strip under each lower eyelid and measure the quantity of tears secreted in five minutes a young normal person more than more than 50 millimeter of each

Paper strip whereas in sugar syndrome it’s less than five millimeter why did i say young normal person it’s because we have to remember that with aging naturally the eyes becomes more dry and therefore older persons sometimes moisten less than 50 millimeter another test of dry ice could be to use slit lamp this test is called tear film breakup time and we stay in

The corner with eye drop dies like the fluorescent or listening green or rose bengal then we ask the patient to blink and we measure the time required for the dry spot to appear on the corner surface of the blinking dry spots occur about 10 seconds off the blinking in normal persons and after 10 seconds an urge to blink again is triggered but when the tear film

Breakup time is less than the blink rate so less than 10 seconds the symptoms of dry ice appear and the treatment for this dry ice can be avoiding drugs that cause dryness for example the anticholinergic theoretics antihistamines and antidepressants we can use artificial tears or lubricant drops like the methyl cellulose or hypermellos we can stimulate lubrication

Of diet locally with cyclic amp or c plus 0.2 percent olive solution only to stimulate systemically with the pillar carpin or savvy melon in severe cases we can put tiny plucks placed in the tear drainage ducts or it can go so far that we have to make a corneal transplantation the dry mouth we’re also called excelostomy of more than three months is also an important

Sign of certain syndrome and this patient is saying that you have daily dry mouth with difficulty of chewing swallowing and even a taste of food is not as good as before so which exocrine glands are destroyed here it’s the cellular glands that like the parity the submandibular the sublingual and minor salivary glands the treatment for dryness of mouth is to drink

Water daily to help swallowing for example we can stimulate the cellular glands locally by sugar-free gum lows and gas or mouthwash containing carboxymetocellulose we can also stimulate salivary glands systemically with drugs like pillow carbine or seven melon as i mentioned before we can avoid drugs that decrease the cellular glass secretion like the anticholinergic

Antidepressants or antihistamines the patient also have tooth decay which can be prevented by regular dental visits with topical fluoride application or simply by keeping a good oral hygiene after each meal she is complaining of secondary candidate infection in this case we give topical nystatin or los angeles or clotrimazole stones can appear in the salivary duct

Which have to be removed to preserve the salivary tissue so i asked the same thing here as i did for i is there any other disease or cause of dry mouth yes there can be viral infections drugs like antihistamine anticholinergic antidepressants anti-hypertensive all these as i mentioned psychological factors irradiation diabetes mellitus or just trauma what test do

We need for dry mouth we can measure the saliva production which will be low in sugar syndrome meaning less than 1.5 milliliter in 15 minutes we can also do technique like cellular synthetic scanning or celography except dryness the patient may present with enlarged parity glands which are smooth firm and rarely painful unless there are there is an obstruction or

Infection so if the pair of the glands are persistently firm we need to rule out the lymphoma if it’s painful with infection we need to apply local wet heat compressors antibiotics and analgesics so we have to think of any other diseases that can cause enlarged piratedness meaning we make a differential diagnosis again so in large part of the glands can be caused

By what viral infections like mumps influenza epstein bar coccyx a acetomegalovirus and even hiv it can be caused by amyloidosis sarcoidosis metabolic problems like diabetes mellitus hyperlipoprotonemias chronic pancreatitis hepatic cirrhosis so even endocrine problems like the acromegaly and gonadal hypofunction can cause enlarged participants until now we have

Only looked at the exocrine lymphoma but there are also extra glandular symptoms and i will mention them in decreasing order so the most common is of algias and arthritis we treat it with hydroxychloroquine or methotrexate and prednisolone reynolds phenomenon can be seen and is treated with nifedipine and gloves which protect from cold lymphadenopathy is this on

The cervical axial region can be seen vasculitis can present itself as rashes on the skin called purpose which can be treated with with glucocorticoids or immunosuppressive agents like the cyclophosphamine kidney diseases like interstitial nephritis kidney stones impaired concentrating ability or just renal tubular acidosis the b carbonate replacement therapy can

Be used for the renal tubular acidosis chronicapper tubular disease risks of non-hodgkin lymphomas can be assessed at 40 times higher here and this can be treated with the anti-cd20 therapy together with the chop regimen which is a chemotherapy regimen consisting of cytophosomet hydroxide on coven and prednisolone so rarely splenomegaly or myositis may be seen so

Until now we have talked a lot about symptom but in order to be sure that this is sugar and syndrome we need to perform some laboratory tests and if we make blood tests we can see leukopenia anemia and increased erythrocyte sedimentation rate and some auto antibodies the auto antibodies or rheumatoid factors and anti-nuclear antibodies called the ssa rho and ssb

La okay except blood tests we can confirm that this is sugar syndrome by taking a biopsy from the lip or more specifically from the minor salivary glands in the book of mucosa so this will show in the microscope that nymphocytes have attacked the salivary glands and there will be many large areas of lymphocytes with the atrophy of the tissue genetic tests can show

An association of sugar syndrome with hladr3 and now we have reached several final part we need to tell if this woman have a primary or a secondary chagrin syndrome so the primer means that you only have sugar syndrome with no other associated disease secondary means that you have sugar syndrome secondarily to another disease the most common associated disease is

Rheumatoid arthritis and there can be many other associate diseases too like systemic lupuserotomatosis systemic sclerosis mixed connective tissue disease vasculitis hashimoto thyroiditis polymyositis primer biliary cirrhosis and chronic autoimmune hepatitis

Transcribed from video
Sjögren's syndrome By Dr. Andras Fazakas