Sjogrens Syndrome Flashcards
define Sjogrens Syndrome?
> most common Systemic autoimmune disorder
> Lymphocytic infiltration
> causing Keratoconjunctivitis sicca (Dry eyes) and Xerostomia
> Additional symptoms present -
> divided as Primary Sjogren’s syndrome (dry eyes and mouth without an underlying connective tissue disease) + Secondary Sjogren’s syndrome (patients have a dry eyes and dry mouth in the background of a connective tissue disorder)
> Multiple specialty involvement in management
what is the aetiology behind sjogrens sydrome?
> oestrogen/ androgen deficiency (older)
viral (younger)
what is the sex demographic of sjogrens sydrome?
9F : 1M
> two age peaks (childhood and post menopausal)
what is the prevalence of population affected?
> 0.5% - 1.56%
what is the percent effected difference in primary and secondary sjogrens syndrome?
60% = secondary
40% = primary
what glands does sjogrens syndrome effect?
> lacrimal gland function (reduced tear production)
> salivary glands (reduced salivary production)
what are the presentations when the lacrimal gland function is reduced to the eyes?
> Dry itchy, burning, gritty eyes
> Redness
> Sensitivity to light, wind
> Swelling
what are the presentations hen there is decreased function of the salivary gland?
> Dry painful mouth
> Loss of taste
> Dental caries
> dorsal aspect of the tongue loses papilla, becomes red/ inflamed/ lobulated
> Dysphagia
> Infections
> Thicker and opaque saliva
> Enlarged and painful salivary glands
what are the extra glandular presentations shown when a patient has sjogren sydrome?
> Fatigue
> Musculoskeletal problems (arthritis, muscle pain)
> Vasculitis
> Renal (kidney problems, abnormal LFTs , hepatits, cirrhosis)
> Neuropathy (numbness) (brain fog)
> Facial pain
> Venous thromboembolism
what is more severe primary or secondary sjogrens syndrome?
> primary as it comes with alot more complications
how do you diagnose sjorgrens syndrome?
> based on a constellation of signs, symptoms and investigations
> classic symptoms, systemic complications
> classification system - 2017 ACR-EULAR classification for primary SS (works for secondary also) = uses both histological and special investigations
how is a patient diagnoses with primary sjorgrens syndrome
> A diagnosis of primary Sjögren’s syndrome is defined as a score of 4 or more on the ACR- EULAR classification .
> These criteria apply to patients who have at least one symptom of ocular or oral dryness or the presence of systemic manifestations suggestive of primary Sjögren’s syndrome
>
what is the first descriptor/ investigation you look for in diagnosing sjogrens syndrome?
> labial gland biopsy
> Patients must have more than 1 focus of inflammation
> a score determined by the number of mononuclear cell infiltrates containing more than 50 inflammatory cells per 4mm squared of minor labial salivery gland obtained on biopsy
> score = 3
how is a labial gland biopsy carried out?
> horizontal incision - between vermillion of lip and sulcus, off centre
> glands may have atrophy if they have sjogrens syndomre
> compilations - risk of numbness, pain bleeding swelling
what is the second item in the classification of sjogrens syndrome you must looked at in diagnosis based on the ACR- EULAR classification ?
> presence of antibodies
> anti- SSA antibodies
measuured in serum - only anti-Ro60 antibodies have been considered; island’s anti-Ro52 antibodies are not specific for sjogrens syndrome
> score = 3
what is the third parameter you must look for in diagnosing sjogrens syndrome based on the ACR-EULAR classification system ?
> ocular staining score of more than 5
> its a score determined by an ophthalmologist on the basis of examination with fluorescein and lissamine green staining, score range = 0-12
> score = 1
how do you test for ocular dryness?
> schemers test
what is the 4th descriptor you must measure in the ACR-EULAR classification to diagnose sjogrens syndrome?
> schemer test of less than 10mm per 5 min (objective evidence of having sjogrens syndrome)
> an assay for measuring tear production inserting filter paper on the conjunctiva in the lower eyelid and assessing the amount of moisture on the paper
> +ve test = less than 10mm, -ve test = more than 10mm
> score = 1
what is the 5th item investigated based on the ACR-EULAR classification to diagnose sjogrens syndrome?
> unstimulated whole salivary flow of less than 0.1ml per minute
> an assay for measuring the rate of salivary flow by collecting saliva in a tube for at least a 5min after the patient has swallowed
> score = 1
why is ultrasound used in helping to diagnose sjogrens syndrome?
> it is not part of the classification
> although is non invasive and can show characteristic sjogrens syndrome glands
> multiple hypo echoic areas
> monitoring progression
what is the management of sjogrens syndrome?
> no cure
> most of the management is aimed at symptomatic relief
> and patient education
> managed by a number of disciplines
> regular dental review
what is the dental review aspect in the management of sjogrens syndrome?
> Dietary advice
> High fluoride products
> Denture hygiene
> Monitor and treat caries/infection
> Monitor for salivary gland infections
what advice should you give your patient who is suffering from sjogrens syndrome?
> Medication should be checked for drugs that cause or contribute to dry mouth
> Avoid agents that cause hyposalivation (alcohol, smoking)
> Frequent sips of water
> Lip lubrication (alalalin based)
> Moist foods
> Humidifier at night
what can you prescribe to your patients suffering from SS?
> Salivary substitutes/sialogues
> Variety of preparations available which include artificial saliva replacements (gels, sprays and mouth rinses) or salivary stimulants (chewing gum, citric acid tablets)
> Ideally should be of neutral pH and contain electrolytes (including fluoride) to correspond approximately to the composition of saliva
> Some of these products are derived from animal products which may be unsuitable for vegetarians and certain religious groups
> Nine artificial saliva preparations included in DPF
what are salivary subitutes?
> sprays or gels
mimic natural saliva
don’t stimulate salivary glands
> saliva orthana (spray) (derived from animal)
biotene
oralbalance
what are salivary sialogues?
> stimulate salviery gland production
options may be acidic
> Chewing gum
> Salivix
> Salivary stimulating tablets (SST)
> Diabetic sweets
what are example of prescribed salivary dialogue?
> Pilocarpine Hydochloride)
> Cholinergic agonist = Residual gland function optimised secretion
> Side effects - a lot of urination
> Contraindications - patients on heart/ respiratory medication
what complications must you manage for in patients with sjogrens syndrome?
> lymphoma - 5% chance
check for persistent areas of swellings
> heart block in the foetus if the mother has primary sjogrens syndrome
