Professional Documents
Culture Documents
Sjogrensyndromecme1 151224004322
Sjogrensyndromecme1 151224004322
Tengku Ezulia
CME Presentation
30th September 2015
ORL Dept, HKL
Sjogren Syndrome
• A chronic autoimmune inflammatory disease which
involves the exocrine glands, significantly decreasing
the quantity and quality of saliva and tears. 1
• 40 and 60 years.
Etiopathogenesis:
• Not known.
• Genes that encode the transporter molecules that move antigens within
the cytoplasm have also been studied. The question of whether a defect in
these molecules might cause antigens such as Ro and La to be more easily
moved to the cell surface has been explored. The transporter molecule
genes have been suggested as possibly being associated with SS
susceptibility.(143) Kaschner et al.(144) looked at immunoglobin genes
and determined that there was a disturbance of B cell maturation and
abnormal editing of immunoglobulin receptors that may contribute to SS.
Mice have demonstrated that chromosomes 1, 4 and 10 hold major
susceptibility loci for autoimmune saladenitis.(145-147) Generally there is
a belief that certain people are more susceptible to autoimmune disease
because of their genetic makeup. Much work is still to be done in this
area.
Summary of flow of inflammation
1. A local inflammation is initiated by a virus or other toxin, a slow wearing down of some lacrimal acinar cell function
because of hormonal changes, ocular surface distress and/or neurological compromise or the arrival of inflammatory
cytokines from the inflamed salivary glands.
2. The inflammation causes a release of pro-inflammatory cytokines by distressed acinar cells and a change in
trafficking of self-proteins, leading to their exposure outside the basal membrane. Dendritic cells are likely to pick them
up.
3. Acinar cells, because of exposure to the pro-inflammatory cytokines, become APCs as they create MHCII molecules
and expose self-proteins such as Ro and La that have now relocated in the cytoplasm because of the confusion within
the cell.
4. The formation of HEVs within the lacrimal gland is a response to the cytokine environment. The cytokines,
chemokines and cell-adhesive molecules on the HEVs of the gland cause migration and homing of lymphocytes and
dendritic cells to the affected gland.
5. Because of the inflammatory environment, local dendritic cells now take up selfantigens and take the message to the
lymph nodes to teach Th cells to recognize the self-proteins. The Th cells return to the lacrimal gland to identify Ro and
La and stimulate the local B cells to become Ig secreting plasma cells. 47
6. As time goes on, the lymphocytic infiltrate organizes itself to maintain a constant inflammation. Specific chemokines
and cytokines promote the local reproduction and prolonged life of B and T cells. This organization is present in a
spectrum from mild organization to actual germinal centres.
The pathophysiology of SS salivary and lacrimal gland inflammation and the subsequent systemic pathology is still in
the early stages of understanding. There is, however, a clearer picture of the T, B, acinar and dendritic cell roles in this
inflammation and the extraordinary influence of the cytokine and hormonal environment
Clinical presentation
• Xerostomia (Dry mouth)
• Candida infections
• Dental caries & decay
• Swollen salivary glands
• Dysphagia – Difficulty or soreness when eating and/or
speaking and notice that their taste is affected.
• Odynophagia – Swallowing difficult and frequent sips of
water are often required
• Hoarseness
• Keratoconjuctivitis sicca (Dry eyes) – feeling of “irritable” or
“gritty eyes”
• Recurrent eye infections.
• Dry skin
• Dry vaginal dryness - painful or difficult sexual intercourse.
Extra glandular manifestations
Systemic manifestations may involve these systems: 4
10. Maternity
SS patients may report a history of miscarriage. Pregnant SS patients
have a higher frequency of the fetal complication of congenital heart
block that relates to the transplacental passage of autoantibodies to
Ro and La. In some patients recurrent miscarriages or vascular
thrombosis are a result of antiphospholipid antibodies.
Diagnostic criteria of SS
American European Combined Criteria (AECC) in 2002
• Artificial tears
• Mild to moderate cases of dry eye can usually be successfully treated with eye drops containing "artificial tears" –
a liquid that mimics tears. These eye drops are available from a pharmacist, without a prescription.
• There are many different types of eye drops, so you can try different brands to find the one that works best for
you. If you're using eye drops regularly (more than three times a day), you should use one that doesn't
contain preservatives. This is because there's evidence that over-exposure to preservatives can damage the
surface of the eye.
• A short-term dose of eye drops containing corticosteroids may be recommended if your eyes become irritated.
However, long-term corticosteroid use isn't recommended because they can cause serious side effects.
• To minimise the chance of experiencing side effects from corticosteroids, you'll be prescribed the lowest effective
dose for the shortest possible time.
• Moisture chamber spectacles
• Wearing glasses reduces tear evaporation by up to 30%, and this effect can be maximised by wearing specially-
made glasses called moisture chamber spectacles. These wrap around your eyes like goggles and help retain
moisture and protect the eyes from irritants.
• Some people used to be embarrassed to wear them, but modern designs look like sports glasses.
• Punctal plugs
• Punctual occlusion is a widely-used technique that seals the tear ducts (into which the tears drain) with small
plugs. This should help keep the eye better protected by tears.
• Temporary plugs made of silicone are usually used first to see if they help. If it does, more permanent plugs can be
used.
Mouth care
• Pilocarpine
• The medicine pilocarpine is often used to treat the symptoms of dry eyes and dry mouth. Pilocarpine stimulates the glands to produce more saliva
and tears.
• Side effects of pilocarpine include:
• hyperhidrosis (excessive sweating)
• nausea
• diarrhoea
• heartburn
• abdominal (stomach) pain
• an increased need to go to the toilet
• For some people, the side effects of pilocarpine are mild. Others find that the side effects outweigh the benefits.
• Don't take pilocarpine if you have asthma or chronic obstructive pulmonary disease (COPD), or if you're pregnant or breastfeeding.
• Hydroxychloroquine
• Hydroxychloroquine has been shown to slow down the immune system's attack on the tear and saliva glands. It can also help reduce any associated
symptoms of muscle pain, joint pain and stiffness.
• You'll need to take hydroxychloroquine for several weeks before you notice any improvements, and it could be six months before you experience the
full benefit of the treatment.
• Side effects are uncommon and usually mild. They include:
• nausea
• skin rash
• loss of appetite
• stomach cramps
• vomiting
• In very rare cases, hydroxychloroquine can damage the retina, affecting vision. You'll probably be asked to attend an eye examination so that your
retina can be checked before you start treatment. Regular eye examinations (usually at least once a year) are also recommended after you begin
treatment.
• Hydroxychloroquine shouldn't be used by breastfeeding women.
Treating other symptoms of Sjögren's
syndrome
• Dry skin
• Several soaps and creams are specifically designed for people with dry skin. Your
pharmacist or GP can advise you.
• Vaginal dryness
• The symptoms of vaginal dryness can be treated using a lubricant. Some women
also use oestrogen creams or hormone replacement therapy (HRT).
• Muscle and joint pains
• Muscle and joint pains can be treated with an over-the-counter non-steroidal anti-
inflammatory drug (NSAID), such as ibuprofen. If this doesn't work, see your GP, as
stronger NSAIDs are available on prescription.
• NSAIDs can increase your risk of developing stomach ulcers and internal bleeding,
particularly if they're taken on a long-term basis.
• If you find swallowing NSAIDs difficult because of your dry mouth, you can try an
NSAID cream that's rubbed into affected joints.
• NSAIDs aren't recommended for pregnant or breastfeeding women, or for people
with pre-existing risk factors for cardiovascular or kidney conditions.
General advice