MINOR SALIVARY GLAND BIOPSY
(LIP BIOPSY)
The minor salivary gland biopsy (MSGB) is commonly referred to as the “lip biopsy.” Another name for it is the labial salivary gland biopsy. This test plays a central role in the diagnosis of Sjogren’s. SSA-positive patients can usually be diagnosed without undergoing a MSGB.
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It is important to take into account the flaws and limitations of this test when interpreting test results. About 20% of known Sjogren’s patients have a negative MSGB.
The reasons for this are described below. A positive MSGB in the context of clinical symptoms strongly supports a Sjogren’s diagnosis. See Myth #5 on the MYTHS ABOUT DIAGNOSIS page to learn why a negative MSGB does not rule out Sjogren’s.
When is the MSGB performed?
MSGB is indicated in SSA-negative ("seronegative") patients who have signs and symptoms of Sjogren’s. Seronegative patients often rely on MSGB to support a Sjogren’s diagnosis. Rheumatologists may decide to order the MSGB for other reasons, such as assessing risk for lymphoma or to look for other diseases such as amyloidosis or IgG4-related disease.
How is the MSGB performed?
Small slivers of tissue are taken from the inside of the lower lip, using local anesthesia. Patients sometimes require a few stitches. This minor procedure removes a few small glands, but not enough to reduce overall salivary flow. If the biopsy is performed by a skilled practitioner, only about 2% of patients experience long-term pain (usually mild) or numbness at the biopsy site. It is important to find out if the person performing the biopsy does this procedure frequently, not just a few times a year.
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Special training/ experience is required to obtain a good lip biopsy sample. The procedure is generally well-tolerated when done by a skilled clinician, usually an oral surgeon or ENT. Equally important is the skill of the pathologist who reads the specimen. The sample should be read by a head and neck pathologist with special training to read these biopsies for Sjogren's. This is more likely to be available in large medical centers and teaching hospitals.
As a veteran of three MSGB procedures,
I can attest to the fact that the procedure was minimally painful.
However, I did have highly experienced clinicians who performed the biopsies.
My second two biopsies, performed by Dr. Ava Wu for the UCSF SICCA study, required no stitches. I was able to eat lunch immediately following biopsy #2 and #3.
~ Sarah Schafer
What does it mean to have a positive MSGB?
A pathologist looks at the sample under a microscope and finds collections (foci) of inflammatory cells that cluster around ductal cells in the salivary gland. A “focus score” is then assigned based on the number of foci (clusters) seen per 4 square mm in a particular sample.
A focus score of 1 or higher is positive (4). Therefore, a focus score of 1 is considered positive, just like a focus score of 4 is positive. A positive test usually, but not always, correlates with dryness. The MSGB may be negative early on, especially when oral sicca lags behind other symptoms (20). A positive MSGB in a patient with clinical Sjogren's features is usually considered a firm diagnosis.
While a positive MSGB usually indicates Sjogren’s, false-positive test results are occasionally seen with Hepatitis C, HIV, post acute Covid infection, graft-versus-host disease, smokers, and in some healthy controls (19).
Advantages Of MSGB
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MSGB is currently the best way to confirm a diagnosis of SSA-negative Sjogren’s. Salivary gland ultrasound is sometimes performed in lieu of MSGB. This requires special training and is not widely available as of 2022.
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The biopsy specimen may provide valuable prognostic information for lymphoma and other direct complications of Sjogren’s (11, 16, 17) Germinal Center (GC)- like structures are seen in about 25 % of positive biopsies. GCs are associated with an increased risk of developing lymphoma (20). Many pathologists do not report GC structures at this time.
Disadvantages & Limitations Of MSGB
The MSGB reflects the salivary gland component of Sjogren’s,
which is just one area impacted by this multisystem disease.
It is inappropriate to call the MSGB the "gold standard" for Sjogren's diagnosis. Sjogren's is a big picture diagnosis.
Learn more about how the limitations of the MSGB (lip biopsy) delayed my diagnosis in the blog post, 12 Reasons People With Sjogren's Don't Get Diagnosed.
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The test is not always available. Seronegative patients may wish to travel to a provider who can perform this procedure, especially if their doctor insists they do not have Sjogren’s due to being SSA- negative.
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While the test should be minimally invasive, provider technique varies. Some patients refuse the procedure, especially when they hear about others who experienced a painful MSGB. It is important to ask about the clinician's experience. Doing a handful of biopsies a year is not enough.
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Sampling procedure is not standardized. Many providers (dentists, ENTs, others) do not take adequate samples due to a lack of consistent training.
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Until recently, no clear standards have been available for reading and reporting biopsy specimens. Mistakes in reading the sample may lead to an incorrect diagnosis (17).
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A negative lip biopsy/MSGB does not rule out Sjogren’s.
False negative results may occur due to problems with the biopsy sample, the reading of the sample, or an inadequate number of representative glands. Early in the disease, inflammation may not have developed into clusters of cells that qualify as foci. Late in the disease, atrophy and scarring may predominate. The focus score may drop to less than 1 as the disease progresses (3, 17).
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Self-Advocacy Resources:
Why is it so hard to get diagnosed?
MYTHS ABOUT DIAGNOSIS
Updated 04-10-2024