Sjogrens Flashcards
what is sjogrens disease
chronic autoimmune disease affecting the salivary and lacrimal glands
what are the 3 classifications of sjogrens disease
sicca syndrome - dry eyes or dry mouth
primary sjogrens - dry eyes and mouth but no connective tissue disease
secondary sjogrens - dry eyes and mouth and another connective tissue disease present e.g RA or SLE
discuss the epidemiology of sjogrens syndrome
predominantly affects females (10:1)
half of those experiencing sjogrens will also have another connective tissue disease
most common age of onset is 50s
discuss the aetiology of sjogrens disease
unknown
possibly genetic
possible environmental - weak association with EBV
discuss the histopathology of sjogrens
lymphocytes infiltrate glands and cluster around ducts replacing acinar cells
(method of acinar destruction unclear)
Over time, lymphocytic infiltrate enlarges and occupies more of the gland
Hyperplasia of ductal epithelium is also seen (possibly due to cytokines released by lymphocytes) which eventually leads to obliteration of duct lumen. This leads to islands of epithelium surrounded by sheets of lymphocytes (salivary lympho-epithelial lesion)
discuss the extra oral manifestations of sjogrens
occasionally bilateral swelling of parotid glands
Ocular effects - gritty sensation in eye, failure of tear secretion, failure of clearance of foreign particles from cornea and conjunctiva
discuss the intra oral manifestations of sjogrens
difficulties eating, swallowing and speaking
mucosa dry, erythmatous, smooth and glazed
dorsum of tongue red/atrophic which varying degrees of fissuring/ lobulation
cervical caries
lymphocytic focus
collection of >50 lymphocytes around a duct
name 3 things included within the exclusion criteria of the ARC EULAR guidelines for sjogrens diagnosis
history of radiation treatment to the head and neck
active hep C infection
AIDS
sarcoidosis
amyloidosis
GVHD
IgG4 related disease
what 2 criteria have a weighted score of 3 in the ARC EULAR criteria of sjogrens
- presence of anti ro antibodies
- labial salivary gland with focal lymphocytic sialadenitis and a focal score >_1
name 2 criteria with a weighted score of 1 in the ACR EULAR diagnosis of sjogrens
occular staining score >_5 in at least one eye
schirmer test <_5mm/ 5 min in at least one eye
unstimulated whole salivary flow test <_1.5ml in 15 min (<_0.1/min)
positive ulstrasound scan added in 2020
why is an ultrasound preferred to a silogram
no radiation used
what would an ultrasound of a gland suffering sjogrens show
‘leopard spot’ appearance
discuss an unstimulated salivary flow test
patient given tube to spit into and volume of saliva is measured over a period of time
>1.5ml in 15 mins indicative of no dry mouth
discuss the schirmer test
specialised paper which absorbs tears
normal tear production sees >5mm in 5 mins
discuss the fluorescin tear film assessment
fluorescin applied to eye and time measured until first dark spot appears
dark spot indicates tear film evaporation
discuss the autoantibodies assoiated with sjogrens disease
anti ro most commonly associated , then anti la
ANA and rheumatoid factor are not associated with sjogrens disease
what is considered the most serious complication of sjogrens
development of a non- hodgkin lymphoma
discuss the risk of lymphoma in a sjogrens patient
sjogrens patients are at increased risk of developing any cancer but a much greater risk of developing a non hodgkin lymphoma in comparison to the general population
how may a salivary lymphoma present
unilateral swelling of a mjor salivary gland
what should be done upon diagnosis of sjogrens due to the higher risk of lymphoma
baseline MRI to be used as a comparison at future lymphoma screens
what treatment may be suggested for a patient who presents with sjogrens before dry mouth has occured
immune modulating treatment in an attempt to halt disease
e.g hydroxychloroquine or methotrexate
discuss the use of salivary stimulants in the treatment of sjogrens
may prove useful for those with dry mouth and salivary deficit
main drug available is pilocarpine - often side effects outweigh benefits
side effects of pilocarpine
sweating, headaches, chills, nausea, palpitations
what toothpaste should patients with sjogrens disease use
5000ppm
