Next stop, we're gonna talk about Sjogren's syndrome.
So let's do a case.
A 54-year-old electrical engineer with a history of seasonal allergies presents with dry eyes.
She states her seasonal allergy symptoms never really went away after last spring,
and seem to have progressed over the past nine months,
despite taking several over-the-counter anti-histamines, nasal sprays, and saline eye drops several times a day.
She reports a 'sandy' sensation in the eyes, mild itching, but denies any eye pain or blurred vision.
No rhinorrhea, no sinus congestion. And no recent viral prodrome.
She reports that she's always drinking water as well because the spring air is, "so dry".
She smokes a pack per day, I'm sure that's not helping. No alcohol or illicit drug use.
And she's sexually active with her male partner. Her sister has Raynaud's disease.
Review of systems; no dyspnea, no fevers, no joint pain. She does report some fatigue and myalgias.
Just a little highlight of Raynaud's disease.
It's always important any time you see in a family history, any reference to Raynaud's disease
or any other autoimmune disease for that matter, to reflect on what other diseases are in that family.
So for Raynaud's disease this family includes things like rheumatoid arthritis,
lupus, systemic sclerosis, and Sjogren's syndrome.
Well, it doesn't guarantee that one of those things will explain her symptoms.
It certainly just important to recognize that her risk of all those conditions
is higher simply by virtue of having a first degree relative with Raynaud's disease.
Alright, so let's jump into our physical exam.
It looks like her vital signs are pretty unremarkable. Pupils are equally rounded, reactive to light.
Extraocular muscles are intact, she does have some mild conjunctival injection,
normal fundoscopic exam however which is good news, no evidence of any blepharitis or inflammation for eyelids,
and the Schirmer test is positive. Let's talk about that for just a moment.
So the Schirmer test is an objective measurement of tear production.
It's performed in a fairly straightforward way.
It's a standardized strip of filter paper, it's placed right on the conjunctival side of the lower lid for five minutes.
And the extend of wetting of that filter paper assesses the extend of tear production.
So if the test is positive, it suggests that she really is not producing as much tears as she ought to be.
Moving on to the oropharynx exam,
she has a dry furrowed tongue supporting the sensation of dry mouth that she was describing.
And she also has enlarged submandibular glands.
Further down she's got no lymphadenopathy but she also has bilateral parotid gland enlargement.
Cardiopulmonary exam, muscular-skeletal exam,
and her skin and nail exam all appear to be unremarkable.
Shown here on the right is just a reminder of what the different glands are that are in the face and in the neck.
So bilateral parotid enlargement, we should just remind ourselves what the differential might be for that.
So of course we've got some viral acute viral syndromes like mumps, the less commonly HIV or CMV,
malnutrition particularly in context of alcohol use disorder, sarcoidosis an infiltrative disease
that's just gonna expand by virtue of granuloma dis-infiltration of the parotid glands,
sialadenitis which is simply inflammation or infection within the parotid gland
so that would be more of a tender swelling and possibly there could be stones in there
that can be contributing to that, Sjogren's disease I think that's the title of this unit, and then malignancy.
You always have to worry about lymphoma and other types of cancers that could infiltrate the parotid glands.
With that let's talk about our differential diagnosis.
First stop, we have sarcoidosis, then allergic conjunctivitis,
Sjogren's syndrome, mumps, and age-related sicca syndrome.
Let's go through each one in turn.
First stop, sarcoidosis; well as I mentioned, it's a multi-system inflammatory disease that can definitely affect the parotids.
It causes parotid inflammation by virtue of granulomatous infiltration and it can even cause a facial nerve palsy.
It does occasionally occur for sure.
Dry eyes however are not very characteristic of this condition.
There weren't much involvement of the eyes aside from the possibility of anterior uveitis or scleritis.
There is a family history of Raynaud's but that's actually not relevant for sarcoidosis
which isn't really considered an autoimmune disease in that sort of way.
So with that in mind, we'll leave it on the list because it can cause parotid gland involvement
but I think we'll move on to the next condition.
Allergic conjunctivitis, well that can cause this dry, sandy, or itchy sensation with her eyes
and it sounds like that's what she's had for many years particularly during the spring season.
However, that wouldn't at all explain the oral symptoms of dryness,
it wouldn't explain the parotid gland enlargement, and it wouldn't explain those myalgias
which give some sense of some sort of autoimmune or system condition going on.
I think we can safely take that one off the list.
Sjogren's syndrome, well it's the name of this video
but also it is most common in middle-aged women which is exactly what she is.
There's a 9:1 gender ratio which also favors women.
So that's certainly supportive of that disease and I'm sure we'll be talking more about it in a few moments.
Mumps, I mentioned there's a few viruses that can cause bilateral parotitis
and it could certainly present like any virus with fevers and myalgias.
Patients symptoms however seem much more indolent than acute infectious process.
She reports that her symptoms have been getting worse over the past nine months.
The ocular symptoms also are not typical features of mumps.
She really don't get dry eyes or that sandy sensation from a viral infection.
So aside from some things that may lean towards mumps, I think we can safely take it off the list.
And lastly, age-related sicca syndrome.
This is a common pattern of dry eyes and dry mouth that's just due to a benign reduction in saliva
and tear production that you can see with progressive age.
You typically see it however in someone a good deal older than our patient.
And then again, it wouldn't explain those myalgias and other associated symptoms.
So I think we can safely take that one off the list too.
Let's take a look at some laboratory data to see if these can guide us with the remaining items on our differential.
First stop, looking at the complete blood count, white count a little on the lower side, hemoglobin same thing,
and the platelet count is just a little bit shy of normal,
so I'll told we have very mild hematologic abnormalities,
creatinine looks okay, liver function test look okay, urinalysis is benign.
And then let's move over to some of the more inflammatory markers.
Her ESR is 69 which is pretty elevated, ANA of 1:160 with a speckled pattern; that's fairly non-specific
but suggests some autoimmune process, double-stranded DNA importantly is absent
which while lupus wasn't really on our list,
it's always good to ensure that you have a double-stranded DNA that's negative if you have an ANA that's positive.
Rheumatoid factor is positive.
I'll remind that you that a lot of things can cause a false positive RF.
So don't automatically be thinking about rheumatoid arthritis.
And then we've got these specific antibodies showing up here.
The anti-Ro and the anti-La. Let's talk a little about those for a moment.
So while the ESR, the ANA, and the RF can commonly be positive in Sjogren's disease,
those tests on the bottom there are a bit more specific for Sjogren's.
In particular the anti-Ro is commonly seen with both lupus but in particular it's more often seen with Sjogren's.
So that being positive is gonna stir us towards that condition.
Anti-La is a little bit less specific and a little bit less sensitive for Sjgorens'
but it does provide some supportive information when it's positive.
So at this juncture with those antibody tests, we can probably safely take sarcoidosis off the differential.
Again, it wouldn't be unreasonable to just get a chest x-ray to be sure
and maybe you can make a case for getting some viral serologies like an HIV, a CMV,
or checking for mumps, that sort of thing.
But burrowing any surprises, it seems that we can confidently make the diagnosis of Sjogren's syndrome.