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
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.
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.
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.