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by Daniel J. Wallace, MD

1. Is there evidence for extraglandular Sjögren’s?

Some people with extraglandular Sjögren’s may have interstitial lung disease, renal tubular acidosis, swollen lymph glands, or inflammatory scarring of the bile ducts (biliary cirrhosis). Being identified with extraglandular Sjögren’s usually warrants systemic immune suppressive therapy with agents such as azathioprine, methotrexate, cyclophosphamide or rituximab. The treating physician should use their tools to screen for the spread of Sjögren’s to new areas with imaging or laboratory testing, which allows one to be proactive and treat the disease early.

2. Screening for lymphoma

Over a 15-20 year period of observation, 8-15% of Sjögren’s patients develop a lymphoma. Screening for symptoms of early lymphoma include asking a patient about swollen glands, fevers, weight loss and new onset of fatigue. A physical examination can detect lymph nodes, evidence for a “wasted” appearance, or an enlarged spleen. I perform a serum protein electrophoresis (a $30 blood test) on my Sjögren’s patients every 6 months. Often, early lymphomas can be detected with the development of an extra protein on this determination, which is known as a “MGUS” or monoclonal gammopathy of uncertain significance. Most Sjögren’s associated lymphomas are of a specific variety known as “MALT” that, if identified early, responds well to treatment.

3. Looking for overlapping Sjögren’s

Sjögren’s patients can have features of other autoimmune conditions such as rheumatoid arthritis, inflammatory myositis, biliary cirrhosis, scleroderma, Hashimoto’s thyroiditis or lupus, while still being “mostly” Sjögren’s. These features may warrant certain anti-inflammatory interventions. This would include corticosteroids for inflamed muscles, drugs that promote more oxygen to dilate the vessels of the hands for individuals with Raynaud’s (often seen with scleroderma or lupus), approaches that halt the development of erosions (bone destruction) with rheumatoid arthritis (e.g., anti-TNFs), ursodiol for biliary cirrhosis, antimalarials for subacute cutaneous lupus rashes in anti-SSA positive patients or thyroid. Identification of a secondary autoimmune overlap can often explain symptoms that may be profound but are not a part of Sjögren’s.

4. Don’t unnecessarily treat Sjögren’s for symptoms that are not related

Sjögren’s patients may have high blood pressure, depression and diabetes, as does 25% of the United States. Medications given for these conditions can make dry eye or dry mouth symptoms more severe. Treating such patients with anti-inflammatory medications or diuretics (water pills) is not advisable. Before altering one’s Sjögren’s medications or their environment, the physician should strive to rule out co-morbidities or co-existing circumstances that may seemingly worsen Sjögren’s symptoms.

5. Screening for head and neck emergencies or ares associated with non-extraglandular Sjögren’s

Patients with Sjögren’s whose disease is confined to the salivary glands, eye, head and neck areas occasionally develop complications, which may mandate emergent treatment. These include acute inflammation of the parotid gland (parotitis, or Mikulicz’s syndrome, with either a stone or focus of inflammation, treated with corticosteroids), corneal ulcerations, blocked salivary ducts (affecting the mouth), and dental caries or abscesses. Most Sjögren’s patients see a dentist 2-3 times a year to get their teeth cleaned, and are often frequent return visitors to their otolaryngologist (ENT doctor). 

In summary, screening for the five features reviewed above, can prevent or promote early treatment of the overwhelming complications with Sjögren’s.

This information was first printed in the Foundation's patient  newsletter for members. 

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Jill, sinus issues were one of my first symptoms of Sjogrens, and now hearing issues , totally due to ENT I have to see more then my Rhuemy, for sinus infections ect...all SJOGRENS RELATED

— Apr 26, 2020

What happens to the 29 million doses of plaquenil in the US stockpile?

— Sep 21, 2020

If you have stable MGUS which does not change, should you still be evaluated for lymphoma? Every six months my rheme doctor at Mayo Clinic run a lot of blood tests, I assume they are checking for this? I also get a physical exam every six months.
Thank you

My husband wants me to go to Mayo. I have been diagnosed with Sjogren’s but I have brain lesions and every symptom of MS since 2004. My neurologist says he feels it is MS but there are a couple key factors that are not there to dx MS. He says Sjogrens can sometimes cause brain lesions. My rheumatologist says he hasn’t heard of that. Have you had better results and answers by going to Mayo?

Becky, I just read yesterday that sjogrens can cause white matter hyperintensities. My neurologist blew that off as signs of aging. I've been trying to get Dx with whatever is wrong with me for 10 years. After my 2nd brain surgery (5 cerebral aneurysms from fibromuscular dysplasia - a connective tissue disease) I started having trigeminal neuralgia symptoms. then three years later the joint pain and fatigue hit me. Then other symptoms hit. I still haven't been Dx, but meeting with a rheumatologist in the next two weeks. I'm not "hunting" for another disease, I just want some of this pain and other symptoms to go away. But I LOVED the Mayo! Best hospital I've ever been to! You should really make an attempt to get there. I went to the one in Jacksonville since I lived 2 miles away from there. Moving back soon.

— Jun 4, 2021

My husband wants me to go to Mayo. I have been diagnosed with Sjogren’s but I have brain lesions and every symptom of MS since 2004. My neurologist says he feels it is MS but there are a couple key factors that are not there to dx MS. He says Sjogrens can sometimes cause brain lesions. My rheumatologist says he hasn’t heard of that. Have you had better results and answers by going to Mayo?

I'm going through this right now. My neurologist told me that a spinal tap will give us a correct diagnosis and that MS and Sjogrens do look similar on an MRI. (I have lesions but my nerves still look okay)

— Nov 20, 2022

Can anyone become a member or is it just for people in the USA? I live in Ireland.


Hi Julia, I've gone around the block with rheumatology, still trying to find one who would try a biological drug just to see if any improvement
I have several chronic white matter hyperintensities on my MRI
How will you manage with such a long wait time

— May 24, 2023

A newbie!! Have so many concerns and questions..I just don't believe this.

— Jun 28, 2023

I have been diagnosed with SS and RA. I’ve had many flares but not as sever as the one I’m recovering from right now. It’s debilitating, I have been suffering for about 2 months now. I told my rheumatologist all of my symptoms and I felt dismissed about them. She won’t prescribe me anything for the muscle pain. I can’t think straight, my body is screaming in pain, I’m exhausted to the point that I need someone else to do the chores at oh me the house. My rheumatologist just keeps prescribing me steroids (I’m on my forth steroid round). Is Mayo the way to go? I need quality and a pain free life to keep living.

Hi Savannah , Sorry what your going through. Because I myself was diagnosed with Sjogrens almost nine months ago . My rheumatologist isn’t treating me and yet I am going back and forth every few week for bloodwork. My body constantly aches, and I mean throbs in main mainly my hips to my feet , and my arms . I get terrible headaches, I never use to get headaches. The doctor asked me is the pain in my joints or muscle ? Ugh , shouldn’t she know . The pain is so excruciating I cannot honestly tell if it’s in my muscles or joints . I cannot hardly sleep at night . I have very dry mouth , where I drink a ton of water a day . I am constantly exhausted when sleeping a good amount of time. And yet this doctor keeps dismissing my illness as if it isn’t anything. Good luck to us dealing with this terrible disease .

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