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About NHL > Types of Lymphoma > Marginal Zone Lymphomas > MALT > Sjögren's Syndrome

Last update: 06/27/2016

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.


2016, technical - full text:

Recent advances in primary Sjogren's syndrome -
Europe PMC Article - Europe PMC http://bit.ly/28Wxhtg

Primary Sjögren’s syndrome, a chronic inflammatory process, is among the most commonly occurring rheumatologic diseases. The clinical hallmark of this disease is exocrine gland dysfunction, resulting predominately in dry eyes and dry mouth. However, the disease often extends beyond the exocrine glands to seriously affect other organs systems, such as the lungs, kidneys, and nervous system. Moreover, patients with primary Sjögren’s syndrome develop non-Hodgkin’s B cell lymphoma at a substantially higher rate than the general population.

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]

Sjögren's and MALT
by Graham, NHL-MALT support group

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: 

bullet dry eyes (xerophthalmia), [1]
bullet dry mouth (xerostomia), 
bullet presence of SS-A and/or SS-B antibodies in the blood, 
bullet a positive lip gland biopsy 
bullet 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 : 

bullet Primary (where it is the only condition) 
bullet 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.


  1. 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
  2. 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
  3. Sjögren's Syndrome Foundation: sjogrens.com 
  4. About - medstudents.com | cibliga.com | .webhealth.co.uk/ | ecureme.com
  5. Describes MALT and SjS in conjunction -  .ncbi.nlm.nih.gov  
  6. 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
  7. 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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