Sjögren Syndrome

by Carlo Raj, MD

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    00:01 Here we have Sjögren Syndrome.

    00:03 After we've discussed Sjögren Syndrome we will then go into the two major inflammatory myopathies and these include polymyositis and dermatomyositis, and by the time we're done you'll have a clear distinction between all three types of myopathies.

    00:19 In Sjögren Syndrome, what it comes down to is dryness.

    00:25 So if the patient is saying, “Doc,” she tells you, “I feel like a sand in my eyes,” that would be involvement of the conjunctiva.

    00:39 “Doc, I miss eating my favorite saltines, I feel so sad. I miss my crackers.” So here’s a patient who has constant irritation taking place in the conjunctiva and the fact that the patient wasn’t able to produce enough saliva to eat her crackers, I say she, cuz autoimmune disease once again is what this is, therefore more prevalent in women.

    01:05 The dryness that’s taking place in the or of the lacrimal glands results in keratoconjunctivitis sicca, whereas if there's a dryness taking place and destruction of the salivary gland result in xerostomia.

    01:18 So now you wanna think of these as being zero lacrimal or tears and zero saliva giving you the presentation that I gave you of irritation in the eyes with sand like irritation; and the fact that the crackers weren’t able to be digested.

    01:38 An autoimmune disease so you have lymphocytic infiltration taking place where? Of all the glands. And by that the two major glands that you're focusing upon will be no doubt the lacrimal, please picture that, no tears, we call this xeropthalmia, may then result in keratoconjunctivitis sicca, not good, and in the parotid, lymphocytic infiltration.

    02:04 That means you don’t have what? Saliva.

    02:06 Now, keep this in mind, whenever there's such lymphocytic infiltration taking place in a chronic nature, especially of let’s say the salivary gland, then there's every possibility that your patient may then develop what? Lymphoma.

    02:19 So in fact, if you have autoimmune disease in general that’ll be let’s say, Hashimoto, of the thyroid gland, then there might be lymphoma taking place of the thyroid.

    02:29 Here a MALToma, mucosa-associated lymphoid tissue.

    02:33 It is a lymphoma taking place in the long term in Sjögren.

    02:36 Can be primary or secondary due to let’s say other connective tissue disease, CTD -- HLA-DR3 associated with primary and HDL-DR4 associated with secondary diseases.

    02:51 Maybe it’s coming from rheumatoid arthritis.

    02:54 Signs and symptoms, and we've talked about a few times now.

    02:57 Dry mouth, inability to appropriately digest or in other words consume simple things like saltine or crackers, and the irritation that’s taking place in the eyes due to the fact that they don’t have the tears, they're like sand in my eyes.

    03:12 The parotid gland will be enlarged and accelerated dental decay because once again you don’t have the saliva for dental carries worst case scenario.

    03:21 Internal organs that could be affected possibly for Sjögren: pancreas, pancreatitis; the lung, interstitial lung disease or fibrosis; renal tubular acidosis, interstitial nephritis; CNS vasculitis could take place, peripheral neuropathy; chronic thyroiditis; or lymphomas as we've talked about, a 40 time increase.

    03:47 So these are the organs that might be affected.

    03:49 Remember, as a rule of thumb, I've given you the major, major issues that you would expect with these diseases, but since these diseases do overlap with overall connective tissue diseases, you can expect therefore to be some of those sharing a signs and symptoms.

    04:07 So those are the things that you're going to picking out however are things that I'm referring to and the markers that I've been repeatedly referring to.

    04:17 Diagnosis, largely kinda clinical. Look for purpura and that would then mean signs of vasculitis or maybe CNS involvement and then more or less might be found for board purposes keep that in mind even though it might not be the most common.

    04:34 However, the combination of the markers of SSA which is then going to be your La A-Ro, and then SSB which is then your La.

    04:46 The combination of the two however is going to be a lot more specific for Sjögren.

    04:51 These are your Anti-Ribonucleoproteins, your RNPs.

    04:55 Also the exam reveals keratoconjunctivitis and your lip biopsy might then find a lymphocytic infiltration of the salivary gland.

    05:05 Let’s have a closer look at Schirmer’s test. This test is done with paper strips which are inserted below the eye to see how much paper is moisturized after five minutes.

    05:17 Normally there are more than 15 mm of moist paper after five minutes.

    05:23 With 9 - 14 mm one would think of mild eye dryness and 4 - 8 mm would be classified as moderate.

    05:31 In severe cases there are less than 4 mm of moist paper.

    05:35 Management, good oral hygiene because you're worried about dental carries.

    05:41 Lubricating eye drops and let me ask you a question, when it comes to secreting in general, is that more of your parasympathetic or sympathetic type of enervation? It would be more of your parasympathetic, wouldn't it? Keep that in mind, I'm gonna give you an agent coming up in a second called Cevimeline.

    05:59 So internal organ involvement treated with immunosuppressant if it comes down to that and as I told you, you would then give a cholinergic agonist such as Cevimeline which is then be responsible for, maybe with this, “Doc, I can now have my favorite crackers!” Compliments of Cevimeline.

    06:18 Welcome to Sjögren.

    About the Lecture

    The lecture Sjögren Syndrome by Carlo Raj, MD is from the course Connective Tissue Diseases. It contains the following chapters:

    • Introduction to Sjögren's Syndrome
    • Signs and Symptoms of Sjögren's Syndrome
    • Diagnosis of Sjögren's Syndrome

    Included Quiz Questions

    1. HLA-DR3
    2. HLA-DR6
    3. HLA-B27
    4. HLA-B5
    5. HLA-B45
    1. Sjogren syndrome
    2. CREST syndrome
    3. Rheumatoid arthritis
    4. Diffuse systemic sclerosis
    5. Systemic lupus erythematosus
    1. Lymphoma
    2. Leukemia
    3. Pancreatic cancer
    4. Colorectal carcinoma
    5. Sarcoma

    Author of lecture Sjögren Syndrome

     Carlo Raj, MD

    Carlo Raj, MD

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