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Oral Medicine > Sjogrens syndrome > Flashcards

Sjogrens syndrome Flashcards

1
Q

Sjogrens syndrome

A
  • systemic multisystem disease
  • autoimmune disease affecting the salivary gland
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2
Q

Types of Sjogrens syndrome

A
  1. Sicca syndrome
    - partial sjogrens findings
    - dry eyes or dry mouth, but not both
  2. SS
    - Primary: no CT disease are found
    - Secondary: CT disease, ie: SLE, Rheumatoid arthritis, Scleroderma (part of other systems)
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3
Q

Autoimmune disease

A
  • intersection of many possible triggers

Triggers
- genetics
- infections
- toxic chemicals
- dietary components

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4
Q

Background of Sjogrens syndrome

A
  • 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
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5
Q

Aetiology of SS

A
  1. 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
  2. Speculative environment
    - EBV association with lymphoma, but interaction unclear
    - weak evidence
    - reactive than other causative
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6
Q

Timeline of Sjogren

A
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7
Q

Consequences of SS

A
  • 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
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8
Q

Immunopathology of SS

A

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
-

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9
Q

Diagnosis of SS

A
  • no single test to give the diagnosis

Different scoring system use:
- American- European Consensus Group (2002)
- ACR- EULAR joint criteria (2016)

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10
Q

American- European Consensus Group (AECG)

A
  • 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
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11
Q

Exclusion criteria for SS

A
  • past H&N radiation tx
  • Hep C infection
  • Acquired immunodeficiency disease
  • Pre-existing lymphoma
  • Sarcoidosis
  • Graft vs Host Disease
  • Use of anti-cholinergic drugs
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12
Q

Summary of American- European Consensus Group (AECG)

A
  1. dry eyes/ mouth
    - subjective/ objective
  2. Autoantibody findings (anti-Ro/ anti-La)
  3. Imaging findings - sialogram/ ultrasound
  4. Radio nucleotide assessment - rare
  5. Histopathology findings
    - labial gland biopsy
    - lymphocytic foci within the salivary tissue
    - duct dilation
    - fibrosis
    - atrophy of acini
  6. needs to have 4 or more positive criteria, as long as wither histopathology/ serology +ve
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13
Q

ACR-EULAR joint criteria (2016)

A
  1. Histopathology findings
    - focus score > 1
  2. Autoantibody findings
    - anti-Ro
  3. Dry eyes/ mouth
    - objective salivary flow
    - Schirmer test
  4. Ultrasound now accepted in 2020
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14
Q

Sjogren in sialogram and Ultrasonography

A
  • snowstorm structure
  • loss of acinar cause holes with no tissues
  • holes will appear visible in ultrasound
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15
Q

AECG Oral and Eye symptoms

A

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

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16
Q

Schirmer test

A
  • 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
17
Q

AECG - oral signs

A
  • abnormal unstimulated whole salivary flow (UWS)
  • abnormal if less than 1.5ml in 15 mins
18
Q

Autoantibodies in Sjogren’s syndrome

A

+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

19
Q

Labial gland biopsy AECG

A

+ve LG biopsy
- predictive test for SS
- before pt present with symptoms, main investigation for SS

  1. collection of >50 lymphocytes around a duct = lymphocytic focus
  2. generalised lymphocytic infiltrate is non-specific sialadenitis
  3. > 1 focus score (FS) consistent with Sjogren syndrome
  4. most diagnostic feature on ACR-EULAR criteria
20
Q

What tests should we do for SS?

A
  1. 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
  2. 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
  3. Labial gland biopsy
    - risk of area numbness
    - informed consent needed

LGB and Anti-Ro may only be the +ve results in early SS

21
Q

Which test will show +ve in SS?

A
  • LGB
  • Anti-Ro
22
Q

Management of SS?

A
  1. 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
  2. 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
23
Q

Complications of SS

A
  1. Effects of oral dryness
    - caries risk, denture retention, infections, functional issues -> speech/ swallow
  2. Salivary enlargement - sialosis
    - can occur anytime -> usually permanent
    - reduction surgery, but not advisable, especially if pt has diabetes
  3. 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

Oral Medicine (24 decks)

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