I find it difficult to follow many people living with primary Sjögren's Syndrome (SS) if their organ system involvement is solely ocular and salivary glands. It seems that when I am not prescribing anything, the patient is left with fatigue that is difficult to treat (if at all) and associated fibromyalgia which seems (at least to me) to occur in at least 1/4 or 1/3 of patients with primary SS.
Dr. Art Bookman presented various cases to enhance our understanding, particularly of the new criteria for Sjögren's Syndrome.
He presented the following case:
2 yr history of irritated eyes – dry and blepharitis reported by her eye doctor
Widespread aches and fatigue
Stongly positive ANA
Is this fibromyalgia alone or SS and fibromyalgia?
There are NEW 2015 criteria to classify Sjögren's Syndrome (Shiboski, C) that were presented at ACR 2015 and will soon be published. They mimic other recent criteria in that they utilize a points system where a score of 4 or more indicates SS.
anti-Ro Positive = 3
Schirmer ≤ 5mm in 5 min = 1
Ocular staining score ≥ 5 (or van Bijsterveld score ≥ 4) = 1
Lip salivary gland biopsy with focal lymphocytic sialadenitis and focus score ≥ 1 = 1
Salivary flow rate ≤ 0.1ml/min = 1
Other interesting tidbits about SS:
Focal lymphocytic is SS whereas chronic sclerosing sialadenitis is not SS; A pathologist with an interest in SS is ideal for differentiation between the two.
There is a 6% lifetime risk of lymphoma in SS, especially if germinal centres are present on biopsy – predictive of future lymphoma - MALT lymphoma
If anti-Ro positive in healthy sera, half get CTD (11 of 24 Ro+ got a CTD among 8000 tested)
Blepharitis is a common problem in SS and can cause dry eyes
Wash eye lashes regularly
There are hyaluronic acid eye drops which may be better if failing artificial tears
Moisture guard spectacles (lab glasses) can help dryness
Rarely steroid eye drops (prescribed by an ophthalmologist) can be transiently used for severe dryness with keratitis, etc.
Poor correlation between pathology on salivary gland and sicca symptoms
Don't forget medications that cause dryness
No data but edible oil may help with severe dry mouth: swish oil in mouth for 10 minutes, spit it out and rinse with warm water
Oral pilocarpine works max 4 to 6 hrs (especially if used later in day – supper, bed, etc) and it only works if glands are not totally scarred (you can't get water from a stone or from a scarred gland)
Treat thrush if present
Prevent oral decay: marginal erosions at gum line is the SS type (not specific, can also happen in bulimia) – try chlorhexidine rinse, avoid sugar, frequent dental visits, get adequate fluoride (so no bottled water)!
Dr. Janet Pope is Professor of Medicine at Western University and Division Head of Rheumatology. Dr. Pope's research interests include epidemiologic studies in scleroderma, classification criteria in systemic sclerosis, systemic lupus erythematosus and rheumatoid arthritis.View Full Bio