Sjogrens

Question 1

what is sjogrens disease

Answer 1

chronic autoimmune disease affecting the salivary and lacrimal glands

Question 2

what are the 3 classifications of sjogrens disease

Answer 2

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

Question 3

discuss the epidemiology of sjogrens syndrome

Answer 3

predominantly affects females (10:1)
half of those experiencing sjogrens will also have another connective tissue disease
most common age of onset is 50s

Question 4

discuss the aetiology of sjogrens disease

Answer 4

unknown
possibly genetic
possible environmental - weak association with EBV

Question 5

discuss the histopathology of sjogrens

Answer 5

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)

Question 6

discuss the extra oral manifestations of sjogrens

Answer 6

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

Question 7

discuss the intra oral manifestations of sjogrens

Answer 7

difficulties eating, swallowing and speaking
mucosa dry, erythmatous, smooth and glazed
dorsum of tongue red/atrophic which varying degrees of fissuring/ lobulation
cervical caries

Question 8

lymphocytic focus

Answer 8

collection of >50 lymphocytes around a duct

Question 9

name 3 things included within the exclusion criteria of the ARC EULAR guidelines for sjogrens diagnosis

Answer 9

history of radiation treatment to the head and neck
active hep C infection
AIDS
sarcoidosis
amyloidosis
GVHD
IgG4 related disease

Question 10

what 2 criteria have a weighted score of 3 in the ARC EULAR criteria of sjogrens

Answer 10

• presence of anti ro antibodies
• labial salivary gland with focal lymphocytic sialadenitis and a focal score >_1

Question 11

name 2 criteria with a weighted score of 1 in the ACR EULAR diagnosis of sjogrens

Answer 11

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

Question 12

why is an ultrasound preferred to a silogram

Answer 12

no radiation used

Question 13

what would an ultrasound of a gland suffering sjogrens show

Answer 13

‘leopard spot’ appearance

Question 14

discuss an unstimulated salivary flow test

Answer 14

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

Question 15

discuss the schirmer test

Answer 15

specialised paper which absorbs tears
normal tear production sees >5mm in 5 mins

Question 16

discuss the fluorescin tear film assessment

Answer 16

fluorescin applied to eye and time measured until first dark spot appears
dark spot indicates tear film evaporation

Question 17

discuss the autoantibodies assoiated with sjogrens disease

Answer 17

anti ro most commonly associated , then anti la
ANA and rheumatoid factor are not associated with sjogrens disease

Question 18

what is considered the most serious complication of sjogrens

Answer 18

development of a non- hodgkin lymphoma

Question 19

discuss the risk of lymphoma in a sjogrens patient

Answer 19

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

Question 20

how may a salivary lymphoma present

Answer 20

unilateral swelling of a mjor salivary gland

Question 21

what should be done upon diagnosis of sjogrens due to the higher risk of lymphoma

Answer 21

baseline MRI to be used as a comparison at future lymphoma screens

Question 22

what treatment may be suggested for a patient who presents with sjogrens before dry mouth has occured

Answer 22

immune modulating treatment in an attempt to halt disease
e.g hydroxychloroquine or methotrexate

Question 23

discuss the use of salivary stimulants in the treatment of sjogrens

Answer 23

may prove useful for those with dry mouth and salivary deficit
main drug available is pilocarpine - often side effects outweigh benefits

Question 24

side effects of pilocarpine

Answer 24

sweating, headaches, chills, nausea, palpitations

Question 25

what toothpaste should patients with sjogrens disease use

Answer 25

5000ppm