Taking Control Part 3 - Sjogren's Conference Dr Ann Parke: Systemic Sjogren's Syndrome

Dr Parke has been a huge supporter of Sjogren's Canada almost from the beginning. Maybe it's due to some of her early training at McMaster University in Hamilton and the Canadian link. Now she is a leading practitioner and educator in this field and we were delighted to welcome her to the conference.  Here she is with Sjogren's Society of Canada President and Founder Lee Durdon.

Photo from Sjogren's Society of Canada. Dr Ann Parke on right, President Lee Durdon on left

Dr Parke began her talk by describing Sjogren's Syndrome as a lymphocyte aggressive disease, that preferentially attacks the exocrine glands, ie the glands that have ducts. She gave three other ways to describe it:
1. an "autoimmune exocrinopathy"
2. an "autoimmune epithelitis"
3. Lupus of the mucous membranes

I love to know how illness is described by doctors so that I am not taken unawares in a visit.

There are a number of criteria to be met to get a diagnosis of Sjogren's Syndrome. They include subjective complaints and objective tests of dryness, pathological changes and auto-antibody production.
Dr Parke described a four pronged approach to management of Sjogren's: 
1.  Manage the complications. 
2. Manage the disease
3. Monitor for serious complications
4. Education

As part of management good dental care is a key with frequent visits to the dentist, fluoride and anti-microbial therapy and the stimulation of secretions (saliva) with oral cholinergic agonists like pilocarpine (Salagen) or cevimiline (Evoxac).  This benefits patients by increasing saliva flow and reducing dental caries and oral candidiasis.  Speaking for myself it helps me with my voice and with swallowing. It's easier to talk and I don't choke as much as I did without it.

Salagen pills in natural habitat

Dr Parke says it's an exciting time in Sjogren's Syndrome, with new treatments in development.

Rituxan has been used since 1997 and has a good safety profile in treatment of primary Sjogren's. Orencia is being looked at as a possibility. Dr Parke uses anti-inflammatory agents like hydroxychloroquine, low dose prednisone, methotrexate and anti-BLYS. She said it takes two months to see results from plaquenil. 
Prednisone is good but it is necessary to watch out for side effects. 

One thing she stressed is that TNF inhibitors do not work.   

There was an open label study of Belimumab in SLE (Lupus) that covered 1746 patient years. At the end of the study 50% of the patients wanted to stay on it. That is a very impressive number. Over the long term patients continued to improve and their steroid use decreased.  Since Sjogren's is a cousin to lupus there is a lot of interest in a potential trial of belimumab in Sjogrens.

In the education area we need to raise awareness in patients, their families, health professionals and funding agencies. That's a big task. 

During the round table discussion I heard more facts from Dr. Parke. 

There is an association between PBC (primary biliary cirrhosis)
and Sjogren's Syndrome - some call it dry liver syndrome. The duct/vein/artery is the biliary triad seen in the liver with PBC. 

Happy Liver from PlushAnatomy on etsy.com

There was one funny moment when Dr. Parke answered a question about livedo reticularis and someone misheard her and started to talk about libido. (Quite a jump)  She said livedo can be associated with clotting disorders and anti-phospholipid syndrome, so one more thing to think about since it is also associated with Sjogren's syndrome.

Taking Control Part 2 - 2014 Sjogren's Society of Canada Dr Mahvash Navazesh: Oral Manifestations

The second lecture of the day began with Dr. Mahvash Navazesh, a professor at the Ostrow School of Dentistry at the University of Southern California, introducing herself as the "Spit Queen". The title of her talk was Oral Manifestations of Sjogren's Syndrome  Most people take saliva for granted," she says. "It's one of the least-respected body fluids."

https://student.societyforscience.org/article/spit-power

The level of dryness in the mouth predicts progression of the disease. Teeth show the effects of mineral loss long before the caries develop (2 to 2 1/2 years). To save teeth we need early detection. Saliva can now predict how susceptible we are to decay and which surfaces are at risk.

We learned that 1% of saliva is not water, but is composed of ions, proteins, small organic molecules, enzymes and microorganisms and by-products. Since dental caries is the most common infectious disease we really need the help of saliva to protect our teeth. It can provide buffers to neutralize plaque acids and to promote remineralization of teeth, as well as by cleansing the surfaces and by maintaining a balance between "good guys and bad guys" in the mouth.

By the time dryness is evident people with Sjogren's have already lost 50% of their salivary gland function. If the saliva loss is a result of medication use, once you stop using the medication function returns in two to three months. When the loss is from Sjogren's it takes constant care and lifestyle adjustment to deal with situations like waking up with your tongue stuck to the roof of your mouth or even taking care not to sample any dry food in a store because you might choke. 

Dr Mahvesh Navazesh

Normal oral care is not enough for us. We have to do more and here is a list of good practices from Dr Navazesh's slides:

Dietary counseling
Avoid alcohol, cariogenic snacks and soft drinks
Avoid dry, crisp, crunchy food and snacks
Meticulous oral home care
Daily oral hygiene, including oral prostheses
Avoid alcohol containing mouth rinses
Daily use of fluoride toothpaste and mouth rinse
Regular professional oral evaluation and care (frequency depends on severity and compliance)
Office application of fluoride varnish, gel or mouth rinse
Hydration/lubrication
Keeping lips and mouth lubricated
Saliva stimulation through xylitol gum, sugarless mints
Use of pilocarpine or evoxac to make the glands work harder

Dentists generally "drill, bill and fill" and sometimes I feel like my mouth will be a gold mine but even a great dentist can't guarantee their work when your oral environment is not normal. On the plus side Dr. Navazesh says that there is hope on the horizon, possibly through gene therapy.

Here are some articles that give more details of Dr Navazesh's work:
Salivary dysfunction associated with systemic diseases: systematic review and clinical management recommendations 

Spit Power

Here's the link to Part 1 of the coverage of the 2014 National Conference of the Sjogren's Society of Canada

Taking Control Part 1: 2014 Sjogren's Society of Canada National Conference Dr Arthur Bookman: Sjogren's Overview

The 8th Annual Conference got off to a good start with comments from President and Founder Lee Durdon. She has been responsible for all of the conferences to date and they just keep getting better.

For the last two years the conference has been accredited as Multidiscipline CME/CE so we have started to see more doctors, dentists and hygienists among the attendees.

Lee said that Sjogren's is like an iceberg.

The Titanic reminds me of icebergs

On the surface there does not seem to be much happening, but the person with the condition knows that they have been besieged. Taking control seems impossible, so we hope the education and connection provided by the conference helps patients to develop a sense of optimism and empowerment.

Being able to cope better with this immune system gone wild is an enabling experience.

Dr Arthur Bookman's talk started the day. He called it "More Than a Nuisance". That is certainly a good description of our own personal iceberg experiences with Sjogren's. 

Dr Bookman is the Co-Chair of the Medical Advisory Board of The Sjogren's Society of Canada. This group of experts advises the Board of Directors and recommends scientific and research goals for the Society. You can see the member's names on the website of Sjogren's Canada.

Dr Bookman starts the conference yearly with an always updated "Overview of  Sjogren's Syndrome".  Of the patients seen at the Sjogren's Clinic that he oversees, 76% had a delayed diagnosis, most often because the healthcare professionals they saw were unfamiliar with the disease. This is the case even though most patients had already seen four professionals before being diagnosed. With numbers like that it is easy to see why our Society feels that increasing awareness is so important.

Dry eye and mouth is worse than it sounds

Dr Bookman also told us that 70% of clinic patients have dry mouth, 60% have dry eye. The diagnosis of secondary sjogren's syndrome may on the way out. Now patients with another autoimmune disease like rheumatoid arthritis, are described as having Sjogren's as well as RA.

One tip he gave us was to massage the salivary glands daily to avoid having them become painful and swollen. His instructional slide for this was by Dr Ava Wu, like this one on the Sjogren's Foundation site.

One hopeful sign for the future is a small trial that seemed to be successful. It describes an allogeneic mesenchymal stem cell treatment that had good results and no adverse events.

There's always a mouse - NOD or ICR?

Sjogren's is a medical syndrome with no one defining element or gold standard test. There are 6 criteria including dryness of eyes and mouth and 4 of them have to be met for a diagnosis. One of those 4 must be a salivary gland biopsy or a blood test for the Ro (SSA) or La (SSB) antibodies.

It is always interesting to hear about the Devins Illness Intrusiveness Scale in which patients with various disease rated the issues they face daily. Sjogren's ended up being almost the same as Multiple Sclerosis in terms of effect on quality of life.

Dr. Bookman at the round table discussion


Through trying to find Dr Ava Wu's slide for salivary gland massage I found this, in case you want to read about Sjogren's at a high level.
Sjogren's Syndrome book chapter  by Dr Ava Wu, Dr John Whitcher and Dr Troy Daniels
Head and Neck Manifestations of Systemic Disease  It's Chapter 2.

Links to Sjogren's posts from the past:

Part 6. Immune System Gone Wild. Dr MiriamGrushka and Dr Lisa Prokopich

Part 5 Immune System Gone Wild Dr Susan Abbey 

Part 4. Immune System Gone Wild. Dr Carl Laskin and Dr Izchak Barzilay 

Part 3 Immune System Gone Wild: SjogrensNational Conference 2013 Dr Bruce Jackson 

Part 2 Immune System Gone Wild. Sjogren's National Conference Dr.Papas

Part 1 Immune System Gone Wild Sjogren's National Conference 2013, Dr. Arthur Bookman 

Sjogren's makes Life More Complicated

2012 National Patient Conference

Sjogren's Products