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
