Sjogrens syndrome Flashcards
Sjogrens syndrome
- systemic multisystem disease
- autoimmune disease affecting the salivary gland
Types of Sjogrens syndrome
- Sicca syndrome
- partial sjogrens findings
- dry eyes or dry mouth, but not both - SS
- Primary: no CT disease are found
- Secondary: CT disease, ie: SLE, Rheumatoid arthritis, Scleroderma (part of other systems)
Autoimmune disease
- intersection of many possible triggers
Triggers
- genetics
- infections
- toxic chemicals
- dietary components
Background of Sjogrens syndrome
- 0.2 - 1.2% people have this
- half of these may have other CT disease
- mostly women 10:1
- diagnostic delay due to late presentations
- lifespan not affected
- risk of neonatal lupus in baby if pregnancy (need to have pacemaker)
- systemic involvement
- lungs, kidney, liver, pancreas, BV, nervous system
- general fatigue and chronic pain
Aetiology of SS
- Speculative genetic
- genetic predisposition- runs in families, no specific inheritance
(anti-Ro and anti-La seems genetic- just association)
- low oestrogen risk gives off getting CT disease -> androgens protective
- incomplete cell apoptosis leads to antigens being improperly exposed
- dysregulation of inflammatory process with dendritic AP cells recruiting Band T cell responses and pro-inflammatory cytokines - Speculative environment
- EBV association with lymphoma, but interaction unclear
- weak evidence
- reactive than other causative
Timeline of Sjogren
Consequences of SS
- gradual loss of salivary/ lacrimal gland tissue through inflammatory destruction
- enlargement of major salivary glands: usually symmetrical, painless - usually a late finding
- increased risk of lymphoma (5%), salivary marginal B cell (MALT) lymphoma
- oral and ocular effects of loss of saliva and tears *** caries risk, taste function
Immunopathology of SS
Labial gland biopsy
- blue dots around the ducts are T lymphocytes which attracted to the area and cause gland destruction
- lymphocytic foci and will present as +ve when more than 50 lymphocytes are present as one
- more than 1 foci will consider diagnosis of SS
-
Diagnosis of SS
- no single test to give the diagnosis
Different scoring system use:
- American- European Consensus Group (2002)
- ACR- EULAR joint criteria (2016)
American- European Consensus Group (AECG)
- both subjective and objective findings
- don’t need to have all, but need to have 4 to be declared as SS, as long as Histopathology or Serotology is +ve
Exclusion criteria for SS
- past H&N radiation tx
- Hep C infection
- Acquired immunodeficiency disease
- Pre-existing lymphoma
- Sarcoidosis
- Graft vs Host Disease
- Use of anti-cholinergic drugs
Summary of American- European Consensus Group (AECG)
- dry eyes/ mouth
- subjective/ objective - Autoantibody findings (anti-Ro/ anti-La)
- Imaging findings - sialogram/ ultrasound
- Radio nucleotide assessment - rare
- Histopathology findings
- labial gland biopsy
- lymphocytic foci within the salivary tissue
- duct dilation
- fibrosis
- atrophy of acini - needs to have 4 or more positive criteria, as long as wither histopathology/ serology +ve
ACR-EULAR joint criteria (2016)
- Histopathology findings
- focus score > 1 - Autoantibody findings
- anti-Ro - Dry eyes/ mouth
- objective salivary flow
- Schirmer test - Ultrasound now accepted in 2020
Sjogren in sialogram and Ultrasonography
- snowstorm structure
- loss of acinar cause holes with no tissues
- holes will appear visible in ultrasound
AECG Oral and Eye symptoms
Oral
- daily feeling of dry mouth for >3 months
- recurrent swelling of SG as an adult
- frequently drink liquid to aid swallowing dry food
Ocular
- persistent troublesome dry eyes for > 3 months
- recurrent sensation of sand/ gravel in eyes
- tear substitutes use> 3 times/ day
Schirmer test
- place into lower eyelid
- 5mm in 5 mins
- lack of tear production is usually the lacrimal gland problem
- prefer fluorescein tear film assessment
- optician will then provide correct tx for this
AECG - oral signs
- abnormal unstimulated whole salivary flow (UWS)
- abnormal if less than 1.5ml in 15 mins
Autoantibodies in Sjogren’s syndrome
+ve autoantibodies
- not causative in disease process
- associated with clinical pattern
- antibodies possible without disease -> need clinical and lab findings
Anti-Ro and Anti-La antibodies
- collection of protein found in cell
Other autoantibodies
- other extractable nuclear antigens (ENA) not associated
- ANA and RF are not associated with sjogrens
Labial gland biopsy AECG
+ve LG biopsy
- predictive test for SS
- before pt present with symptoms, main investigation for SS
- collection of >50 lymphocytes around a duct = lymphocytic focus
- generalised lymphocytic infiltrate is non-specific sialadenitis
- > 1 focus score (FS) consistent with Sjogren syndrome
- most diagnostic feature on ACR-EULAR criteria
What tests should we do for SS?
- Look into pt’s mouth
- they will always complain of dryness, having a dry mouth
- early SS pt will not have a dry mouth, hence will not complain of one - Do least harmful test
- unstimulated salivary flow/ Unstimulated whole saliva (UWS) in 15 mins <1.5ml
- Anti- Ro antibody
- Salivary USS (ultrasound scan)
- Baseline MRI of major SG-> useful for future lymphoma screen - Labial gland biopsy
- risk of area numbness
- informed consent needed
LGB and Anti-Ro may only be the +ve results in early SS
Which test will show +ve in SS?
- LGB
- Anti-Ro
Management of SS?
- If patient present with dry mouth and salivary deficit
- low gland function
- oral health: diet, OHI, 5000ppm TP
- symptomatic tx of oral dryness
- salivary stimulant- Pilocarpine-> sweating and palpitations as side effects - If pt presenting early
- no dry mouth yet, but active gland disease
- liaise with rheumatologist as a multisystem disease
- consider immune modulating tx -> hydroxychloroquine, methotrexate
Complications of SS
- Effects of oral dryness
- caries risk, denture retention, infections, functional issues -> speech/ swallow - Salivary enlargement - sialosis
- can occur anytime -> usually permanent
- reduction surgery, but not advisable, especially if pt has diabetes - Lymphoma risk
- salivary lymphoma may present with unilateral gland swelling at any stage
- increased general lymphoma risk too
- screening -> GDP should review
- MRI will then be taken
