Sjögren's Syndrome Flashcards
1
Q
What are the two closely related but distinct diseases related to Sjögren’s syndrome?
A
- Primary Sjögren’s syndrome: comprises dry mouth and dry eyes, not associated with any connective tissue disease
- More severe oral and ocular changes than secondary
- Higher risk of complications than secondary - Secondary Sjögren’s syndrome: Comprises dry mouth and dry eyes associated with rheumatoid arthritis or other connective tissue diesase
- Affects 10-15% of pts with RA
- Affects 30% of pts with lupus erythematous
2
Q
Is primary Sjögren’s syndrome a localised form of disease?
A
No, although pts lack a CT disorder, they have other systemic manifestations including involvement of all exocrine glands and features such as Reynaud’s phenomenon
3
Q
What are the clinical features of Sjögren’s syndrome
A
- Relatively common
- Middle-aged or elderly women mainly affected
- Salivary gland damage is irreversible
- Oral effects
- Ocular effects
4
Q
What are the oral effects of Sjögren’s syndrome?
A
- In early stages, mucosa may appear moist, but salivary flow measurement shows diminished secretion.
In established cases, oral mucosa is obviously dry, often red, shiny and parchment like. Tongue is red, the papillae characteristically atrophy and the dorsal becomes lobulated with cobblestone appearance - Less saliva => changes in oral flora => candida infection
- Discomfort and soreness of the mouth
- Generalised erythema of the mucosa
- Often with angular stomatitis
- Plaque accumulates, and there may be rapidly progressive dental caries
- Difficulties with eating or swallowing
- Disturbed taste sensation
- Disturbed quality of speech
- Predisposition to infection
- The most severe infective complication is suppurative parotitis (hot, tender parotid swelling with red, shiny overlying skin)
5
Q
What are the ocular effects of Sjögren’s syndrome
A
- Failure of tear secretion => dry eyes
- Dried secretions stick to conjunctiva and cornea
- Failure of clearance of foreign particles from the cornea and conjunctiva
- Gritty sensation in the eyes
- Corneal damage and scarring
- Keratinisation and loss of goblet cells in conjunctiva
- Abrasions, ulcers and inflammation
- Risk of impairment or loss of sight
6
Q
What is the aetiology of Sjögren’s syndrome
A
- Unknown cause, but genetic predisposition exists and environmental triggers are suspected
- Autoimmune attack on all exocrine glands, including those of skin, vagina, lung and pancreas, although these other sites rarely cause significant problems clinically
7
Q
What is the pathology of Sjögren’s syndrome
A
- Lymphocytes infiltrate the glands and cluster around small ducts, proliferate and gradually replace acinar cells
- Over many years, the lymphocytic infiltrates enlarge and replace more of the gland
- The ductal cells proliferate to form islands and sheets of cells around the ducts (called epimyoepithelial islands). The overall appearance is called myoepithelial sialadenitis
- Eventually results in destruction of acini and replacement of whole gland by dense lymphocytic infiltrate
- The infiltrate is confined within the gland capsule- doesn’t cross the intraglandular septa
- There is systemic polyclonal B cell activation, producing a variety of autoantibodies that aid diagnosis (particularly Ro (SS-S) and La (SS-B))
8
Q
What diagnostic tests can be done to diagnose Sjögren’s syndrome?
A
- No test is definitive. Many may be required in early disease and the least invasive tests are used first:
- Diminished total salivary flow rate
- Unstimulated: spit in cup for 10 mins. 1-2ml/min good; <0.2ml/min bad - Diminished tear secretion and ocular effects
- Schirmer test: filter paper strip tucked under lower eyelid to measure tear production - Blood tests
- Raised immunoglobulin levels and erythrocyte sedimentation rate
- Antibody screen, esp rheumatoid factor and SS-A and SS-B
- Circulating CD4+/CD8+ lymphocyte ratio - Sialectasis on sialography or ultrasound
- Labial salivary gland biopsy showing periductal lymphocytic infiltrate
- 6-8 glands required => 4mm^2 of tissue
- Only if definitive diagnosis is still not possible
9
Q
What is the management for Sjögren’s syndrome
A
- Support and reassurance
- Ophthalmological investigation for keratoconjunctivitis sicca (symptomless)
- Refer to specialist if CT tissue disease is untreated
- Check for any associated drug treatment contributing to dry mouth
- Alleviate dry mouth:
1. Sips of water
2. Saliva substitutes - Control caries
1. Avoid sweets and check diet for excess sugar:
2. Sugar-free gum
3. Maintain good OH
4. Fluoride appciation
5. CHX rinses - Monitor for mucosal candidosis
- Treat difficulty with dentures symptomatically
- Observe regularly for possible development of ascending parotitis or lymphoma
10
Q
What are the complications of Sjögren’s syndrome?
A
- Significant risk of salivary gland and extra salivary lymphomas
(Lymphomas of B cell type, usually of the low-grade MALT (muscoa associated lymphoid tissue) type => good prognosis - Depression is a common consequence: dry and sore mouth/eyes is distressing
11
Q
How does the Challacombe scale work and what are the features to look out for?
A
- Works as an additive score of 1 to 10 and each feature scores 1
- Features:
1. Mirror sticks to buccal mucosa
2. Mirror sticks to tongue
3. Saliva frothy
4. No saliva pooling in FOM
5. Tongue shows generalised shortened papillae
6. Altered gingival architecture
7. Glassy appearance of oral mucosa, especially palate
8. Tongue lobulated/fissured
9. Cervical caries (more than two teeth)
10. Debris on palate or sticking to teeth
12
Q
What management does an additive score of 1-3 on the Challacombe scale indicate?
A
- May not need tx
- Sugar free chewing gum for 15 mins twice daily
- Attention to hydration
13
Q
What management does an additive score of 4-6 on the Challacombe scale indicate?
A
- Sugar free gum or simple sialogogues may be required
- Need for further investigations if reasons for dryness not clear
- Saliva substitutes and topic fluoride may be helpful
- Monitor at regular intervals for decay
14
Q
What management does an additive score of 7-10 on the Challacombe scale indicate?
A
- Saliva substitutes and topical fluoride needed
- Cause of hypo salivation needs to be ascertained and Sjögrens Syndrome excluded
- Pt monitored for changing symptoms and signs with possible specialist input if worsening
15
Q
What is the difference between xerostomia and hyposalivation
A
- Xerostomia is the subjective complaint of dryness
- Hyposalivation is the objective dryness
