Sjogren's syndrome Flashcards

1
Q

What is Sjogren’s syndrome?

A

An autoimmune disease affecting the salivary glands

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2
Q

What are the three classifications of Sjogren’s syndrome?

A
  • Sicca syndrome - dry eyes or dry mouth (partial)
  • Primary - not associated with connective tissue disease
  • Secondary - associated with connective tissue disease
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3
Q

What 3 connective tissue disease are associated with secondary sjogren’s syndrome?

A
  • Systemic Lupus erythematousus
  • Scleroderma
  • Rheumatoid arthritis
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4
Q

What factors may be associated with the development of an autoimmune disease?

A
  • Genetics
  • Diet
  • Infections
  • Drugs
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5
Q

What are 3 symptoms of Sjogren’s syndrome?

A
  • Drys eyes and mouth
  • Fatigue
  • neuropathy , joint pain
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6
Q

What is the incidence of Sjogren’s syndrome?

A

0.2 - 1.2 % , mostly affecting women

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7
Q

What risk is associated with pregnant women with Sjogren’s ?

A
  • risk of neonatal lupus in the baby
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8
Q

What are 2 aetiology factors of Sjogren’s syndrome

A
  • genetic - associated with anti-Ro and anti-La antibodies , low estorgen
  • environmental - may be associated with EBV
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9
Q

Why is Sjogren’s syndrome is diagnosed late?

A

Due to late presentation of symptoms

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10
Q

How do Sjogren’s patient’s loose salivary/lacrimal gland tissue?

A
  • through inflammatory destruction
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11
Q

What other 3 consequences are associated with Sjogren’s syndromes other than gradual loss of salivary and lacrimal gland tissue?

A
  • Enlargement of major salivary glands - symmetrical and painless
  • Increased risk of lymphoma (5%)
  • Oral and ocular effects due to loss of saliva and tears
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12
Q

What types of inflammatory cells attack the salivary and lacrimal glands in Sjogren’s syndrome?

A

T lymphocytes

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13
Q

What is the name of the lymphoma that is caused in the salivary glands due to Sjogren’s?

A

Salivary marginal B-cell (MALT) lymphoma

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14
Q

2 histological signs of Sjogren’s

A
  • more than 1 lymphocytic foci (50 lymphocytes in one area)
  • Damage to acini cells
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15
Q

What are the two different systems used to diagnose systems?

A
  • American-European Consensus Group (2002)
  • ACR-EULAR joint creteria
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16
Q

How many of the criteria of AECG does a patient need to be positive for a diagnosis of Primary Sjogren’s?

A

4 criteria met including Histopathology or Serology being positive

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17
Q

Give 3 exclusion criteria on AECG?

A
  • Hepatitis C infection
  • Post head and neck radiotherapy
  • Sarcoidoses
  • Acquired immunodeficiency disease
18
Q

List the 5 AECG criteria

A
  • Dry eyes/mouth - subjective or objective
  • Autoantibody findings
  • Imaging findings
  • Radio-nucleotide assessment
  • Histopathology findings
19
Q

According to ACR-EULAR , what is the most diagnostic predictor of Sjogren’s syndrome?

A
  • Labial gland biopsy with more than 1 focus score
20
Q

Other than labial gland biopsy what 4 tests is mentioned in the ACR-EULAR 2016 to diagnose Sjogren’s ?

A
  • Autoantibody tests for Anti-Ro
  • unstimulated whole salivary flow
  • Schrimer’s test
  • Salivary Ultrasound scan
21
Q

How much does positive histopathology findings and anti-ro weigh in ACR-EULAR ?

A

each weigh 3

22
Q

How much does positive objective salivary flow and schrimer’s , and ultrasound test weigh in ACR-EULAR ?

A

1

23
Q

What Feature is present in Sjogren’s sialogram ?

A

Snowstorm, due to loss of acini causing holes which are penetrated by the contrast dye

24
Q

What feature is present in Sjogren ultrasound?

A

Leopard spot appearance

25
Q

Why is Ultrasound preferred over sialogram?

A

To reduce patient exposure to ionising radiation

26
Q

Give the 3 oral symptoms in the AECG criteria?

A
  • Daily feeling of dry mouth for more than 3 months
  • Recurrent swelling of salivary glands as an adult
  • Frequently drink liquid to aid swallowing dry foods

Presence of 1 means positive

27
Q

Give 3 Oclular symptoms mentioned in AECG criteria?

A
  • Persistence dry eyes for more than 3 months
  • Recurrent sensation of sand in the eyes
  • Use of tear substitutes more than 3 times a day

Any 1 is positive

28
Q

What reading is abnormal is Schrimer test

A

<5mm wetting in 5 minutes

29
Q

What other test can be an alternative to Schrimer’s test?

A

Fluorecein Tear film assessment

30
Q

What is the positive reading for unstimulated whole salivary flow?

A

less than 1.5 ml in 15 mins

31
Q

What are the 2 most common anti-bodies associated with Sjogren’s syndrome?

A
  • Anti-La
  • Anti-Ro
32
Q

What is this?

A

Collection of more than 50 lymphocytes around a duct

33
Q

Which 4 tests would you do first to investigate Sjogren’s syndrome?

A
  • Unstimulated whole salivary flow test
  • Anti-ro antibody test
  • Salivary ultrasound scan
  • Baseline MRI when it is diagnosed
34
Q

What risk is associated with labial gland biopsy?

A

Skin numbness around the area

35
Q

What 2 tests would be the only ones positive in early Sjogren’s?

A
  • Labial gland biopsy
  • Anti-ro antibody test
36
Q

2 management options for patient with a dry mouth?

A
  • OHI and high fluorife toothpaste
  • Pilocarpine to stimulate saliva
37
Q

If a patient get diagnosed with Sjogren’s syndrome in early stages with no dry mouth yet , How would you manage this patient?

A
  • Liaise with rheumatologist
  • Consider immune modulating treatment
38
Q

What 2 immune modulating drugs can be given to a Sjogren’s syndrome patient?

A
  • Hydroxychloroquine
  • Methotrexate
39
Q

What can be 4 complications of oral dryness in the patient mouth?

A
  • High caries risk
  • Denture retention problems
  • Risk of infection
  • Difficulty swallowing
40
Q

What feature of salivary glands may indicate lymphoma?

A

Unilateral swelling