Graham's post to NHL-MALT (within)
in support of a
member's questions about this syndrome
is a great example of patients providing useful and balanced
information in response to patients in need.
Sjögren's
(pronounced SHOW GRINS) syndrome is a common autoimmune diseases,
in which the body's
immune system mistakenly attacks (creates antibodies against) its
own moisture producing glands.
The classic signs of the Sjögren’s syndrome includes enlargement
of the parotid glands with mucosal dryness manifest by dry mouth
(xerostomia) and dry eyes (xerophthalmia). - medstudents.com
The estimated prevalence of
mucosa-associated lymphoid tissue (MALT) lymphoma in patients with Sjogren
syndrome was 5.8%. [2]
Janet wrote: My only symptoms, if they are symptoms, are chronic low white
count and lumps under my chin which are probably the parotid.
This is what I have figured out via my Sjogren's
Syndrome, I'm not medically trained so there may be a few lies in here
:-)
There are different types of salivary gland. The
parotids are at the back of the jaw, just forward of each ear. There
are also mandibular glands (which are probably the ones you've
noticed) and I think the sublingual gland is in the area under the
tongue. Together these form the major salivary glands. There are then
a large number of minor salivary gland located around the mouth in
various places such as behind the bottom lip. Picture
it
The major and minor glands produce different types
of saliva - thin fluid types and thick mucosal types. A dysfunction in
these glands can disrupt the normal balance of saliva, and in my case
loss of parotid function (which produces thin saliva) means I only
produce the sticky stuff.
I also have chronic dry mouth and dry eyes, but I have been tested
for Sjogren's (blood test) and that test is negative.
Different clinicians have their own view on how to
diagnose Sjogren's Syndrome and what it entails. The definition I
understand to be most widely accepted is a number of the following
holding true:
- dry eyes (xerophthalmia), [1]
- dry mouth (xerostomia),
- presence of SS-A and/or SS-B antibodies in the
blood,
- a positive lip gland biopsy
- and an underlying rheumatic condition.
In my case I have dry eyes (kcs), dry mouth,
positive antibodies, positive biopsy and the possibility of peripheral
neuropathy.
I have heard that "Secondary Sjogren's" (caused by
something else) can only be diagnosed with a biopsy. Does
anyone have input on that?
By my interpretation, it goes like this. There are
two types of Sjogren's Syndrome - hereafter abbreviated to SjS :
- Primary (where it is the only condition)
- Secondary (where it is found in the presence
of a connective tissue disorder, or in the absence of other
autoimmune disorders)
I am currently believed to be a primary, and I
*understand* that Primary SjSs are more susceptible to MALT and certain
other lymphomas. [Get Reference] Secondary SjS would typically have SjS in conjunction
with rheumatoid arthritis, lupus (SLE), fibromyalgia and/or other
similar conditions. Both primary and secondary SjS can have central
nervous system (CNS) implications, bringing on things like the sjogie
"brain fog" which can't really be described easily. [Get
Reference]
Diagnosis of the presence of SjS is as above and is
normally carried out by rheumatologists following a careful capture of
your history, doing blood work, testing tear production via a
Schirmer's Test (blotting paper in the eye) and is usually confirmed
by taking a sample of minor salivary glands from the inside of the
lower lip - the so-called lip biopsy - and examining it under the
microscope.
Once you have a SjS diagnosis the question of
whether it is secondary is dependant on tests for other rheumatic or
connective tissue disorders. The testing process for these will vary.
So, the diagnosis of secondary SjS isn't confirmed by biopsy as such,
but the test for SjS as a whole usually is. Which "branch"
of SjS you have is confirmed by presence of other underlying
conditions.
Do you think my dry mouth (really, really dry and annoying) could
be related to the MALT or the lumps under my chin? I am getting
to the point where I need something for the dry mouth.
Dry mouth is a common symptom of SjS (and I know
exactly what you mean, I have the same) but this can also be caused by
a wide number of other situations including caffeine and medication.
If you have dysfunction in your salivary glands, that will almost
certainly influence a dry mouth condition.
You may also experience other problems with dryness. Common examples would be dryness of the airways and digestive tract (leading to a distinctive cough and sensations of a "knot" in the Adam's Apple area), dry skin and scalp, and vaginal dryness or discomfort.
The crucial part of your
question - whether MALT is implicated - depends on whether you have
MALT in those glands or whether they are swollen as part of the normal
SjS disease process. For me, that had to be determined by a PET
scan, fine needle aspiration and ultimately a surgical biopsy to
remove parotid tissue for microscopic analysis and histology. If your
glands are free of lymphoma then it's likely that your dry mouth
condition is due to other causes.
I would be tempted to suggest you approach a
rheumatologist for a further discussion on SjS and perhaps be
re-tested. It can be difficult to spot and is often hidden by other
conditions or can masquerade as them. Negative blood work in itself
might not be sufficient to draw a conclusion either way; you may need
further tests. If you have dry eyes, dry mouth and swollen glands then
I would certainly be leaning in that direction but I stress that I am
not medically qualified.
As to management of the dry mouth condition, there
are a number of moisture replacement therapies available. I personally
use and can recommend the Biotene range which includes toothpaste,
mouthwash and an oral gel. Between these products I have dramatically
improved my dryness situation and night times are much more bearable.
Incidentally I have no commercial interest in this range, I'm just a
consumer.
More information on SjS can be found on http://www.dry.org/
or via the yahoo groups lists SS-L and TalkSjo.
Information on Biotene is available from http://www.laclede.com/.
Another useful link and discussion of primary SjS versus lymphoma is
http://www.medstudents.com.br/reumat/reumat4.htm
which states: "Malignant or pseudomalignant lymphoproliferation may be a prominent part of the illness, specially in primary Sjögren’s syndrome."
I hope some of this is of interest.
~ Graham in England
One of the 10% male population of SjS people - 90% are female and are
generally around menopausal or postmenopausal age.
Resources:
-
DRY EYES: Albumin as a tear supplement in the
treatment of severe dry eye.
Br J Ophthalmol. 2003 Oct;87(10):1279-83. PMID:
14507766 | Related
abstracts
-
MALT AND Sjögren's: Clinical
and imaging findings of lymphoma in patients with Sjogren
syndrome.
J Comput Assist Tomogr. 2003 Jul-Aug;27(4):517-24. PMID:
12886135 | Related
abstracts
-
Sjögren's Syndrome Foundation: sjogrens.com
-
-
Describes MALT and SjS in conjunction - .ncbi.nlm.nih.gov
-
CASE REPORT: Improvement in Sjogren's syndrome following
therapy with rituximab for marginal zone lymphoma.
Arthritis Rheum. 2003 Jun 15;49(3):394-8. No abstract available. PMID:
12794796
-
Images of lip biopsies etc - med.ohio-state.edu
- PDFf
Also "There is 40 times increased risk of lymphoma in a patient with Sjogren's Syndrome".
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