Sjogren's syndrome Flashcards
What is Sjogren’s syndrome?
An autoimmune disease affecting the salivary glands
What are the three classifications of Sjogren’s syndrome?
- Sicca syndrome - dry eyes or dry mouth (partial)
- Primary - not associated with connective tissue disease
- Secondary - associated with connective tissue disease
What 3 connective tissue disease are associated with secondary sjogren’s syndrome?
- Systemic Lupus erythematousus
- Scleroderma
- Rheumatoid arthritis
What factors may be associated with the development of an autoimmune disease?
- Genetics
- Diet
- Infections
- Drugs
What are 3 symptoms of Sjogren’s syndrome?
- Drys eyes and mouth
- Fatigue
- neuropathy , joint pain
What is the incidence of Sjogren’s syndrome?
0.2 - 1.2 % , mostly affecting women
What risk is associated with pregnant women with Sjogren’s ?
- risk of neonatal lupus in the baby
What are 2 aetiology factors of Sjogren’s syndrome
- genetic - associated with anti-Ro and anti-La antibodies , low estorgen
- environmental - may be associated with EBV
Why is Sjogren’s syndrome is diagnosed late?
Due to late presentation of symptoms
How do Sjogren’s patient’s loose salivary/lacrimal gland tissue?
- through inflammatory destruction
What other 3 consequences are associated with Sjogren’s syndromes other than gradual loss of salivary and lacrimal gland tissue?
- Enlargement of major salivary glands - symmetrical and painless
- Increased risk of lymphoma (5%)
- Oral and ocular effects due to loss of saliva and tears
What types of inflammatory cells attack the salivary and lacrimal glands in Sjogren’s syndrome?
T lymphocytes
What is the name of the lymphoma that is caused in the salivary glands due to Sjogren’s?
Salivary marginal B-cell (MALT) lymphoma
2 histological signs of Sjogren’s
- more than 1 lymphocytic foci (50 lymphocytes in one area)
- Damage to acini cells
What are the two different systems used to diagnose systems?
- American-European Consensus Group (2002)
- ACR-EULAR joint creteria
How many of the criteria of AECG does a patient need to be positive for a diagnosis of Primary Sjogren’s?
4 criteria met including Histopathology or Serology being positive
Give 3 exclusion criteria on AECG?
- Hepatitis C infection
- Post head and neck radiotherapy
- Sarcoidoses
- Acquired immunodeficiency disease
List the 5 AECG criteria
- Dry eyes/mouth - subjective or objective
- Autoantibody findings
- Imaging findings
- Radio-nucleotide assessment
- Histopathology findings
According to ACR-EULAR , what is the most diagnostic predictor of Sjogren’s syndrome?
- Labial gland biopsy with more than 1 focus score
Other than labial gland biopsy what 4 tests is mentioned in the ACR-EULAR 2016 to diagnose Sjogren’s ?
- Autoantibody tests for Anti-Ro
- unstimulated whole salivary flow
- Schrimer’s test
- Salivary Ultrasound scan
How much does positive histopathology findings and anti-ro weigh in ACR-EULAR ?
each weigh 3
How much does positive objective salivary flow and schrimer’s , and ultrasound test weigh in ACR-EULAR ?
1
What Feature is present in Sjogren’s sialogram ?
Snowstorm, due to loss of acini causing holes which are penetrated by the contrast dye
What feature is present in Sjogren ultrasound?
Leopard spot appearance
Why is Ultrasound preferred over sialogram?
To reduce patient exposure to ionising radiation
Give the 3 oral symptoms in the AECG criteria?
- 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
Give 3 Oclular symptoms mentioned in AECG criteria?
- 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
What reading is abnormal is Schrimer test
<5mm wetting in 5 minutes
What other test can be an alternative to Schrimer’s test?
Fluorecein Tear film assessment
What is the positive reading for unstimulated whole salivary flow?
less than 1.5 ml in 15 mins
What are the 2 most common anti-bodies associated with Sjogren’s syndrome?
- Anti-La
- Anti-Ro
What is this?
Collection of more than 50 lymphocytes around a duct
Which 4 tests would you do first to investigate Sjogren’s syndrome?
- Unstimulated whole salivary flow test
- Anti-ro antibody test
- Salivary ultrasound scan
- Baseline MRI when it is diagnosed
What risk is associated with labial gland biopsy?
Skin numbness around the area
What 2 tests would be the only ones positive in early Sjogren’s?
- Labial gland biopsy
- Anti-ro antibody test
2 management options for patient with a dry mouth?
- OHI and high fluorife toothpaste
- Pilocarpine to stimulate saliva
If a patient get diagnosed with Sjogren’s syndrome in early stages with no dry mouth yet , How would you manage this patient?
- Liaise with rheumatologist
- Consider immune modulating treatment
What 2 immune modulating drugs can be given to a Sjogren’s syndrome patient?
- Hydroxychloroquine
- Methotrexate
What can be 4 complications of oral dryness in the patient mouth?
- High caries risk
- Denture retention problems
- Risk of infection
- Difficulty swallowing
What feature of salivary glands may indicate lymphoma?
Unilateral swelling
