LECTURE 6 (Sjögren's syndrome) Flashcards
What is Sjögren’s syndrome?
An autoimmune disorder that leads to destruction of the salivary and lacrimal glands which leads to problems in the mouth and eyes
What are the main symptoms of Sjögren’s syndrome?
- Dry eyes (Keratoconjuctivitis sicca)
[natural consequence of autoimmune destruction of lacrimal glands + presents as feeling of dirt/debris in eyes] - Dry mouth (Xerostomia)
[hard to chew dry food + develop cavities + bad breath]
What are the “Extraglandular” disease symptoms?
- Xerosis
[dry, scaly skin often occurring in lower extremities and axilla] - Pain in joints (Arthralgias) and inflammation of joints (Arthritis)
- Raynaud’s phenomena
[Ischaemia of hand that gets worse in cold weather due to over-vasoconstriction of blood vessels]
What is the usual demographic for Sjögren’s syndrome?
- More common among women
- Age of onset is usually 40s
ADDITIONAL INFO:
Many elderly patients have “sicca symptoms” (dry mouth, dry eyes) but this is due to old age not Sjögren’s syndrome -> antibody tests + biopsy will appear normal
Describe the Immunology of Sjögren’s syndrome
Lymphocyte mediated (Type IV hypersensitivity disorder)
Explanation: Important clinically because if you biopsy the salivary gland, lymphocytes can be seen “Lymphocytic Sialadenitis” (HALLMARK!!!)
In which syndromes does Sjögren’s syndrome appear secondary to?
- Rheumatoid Arthritis + Lupus (complicates both)
- Primary billiary cirrhosis (autoimmune destruction of bile ducts)
Which antibody tests are relevant to the diagnosis of Sjögren’s syndrome?
- Antinuclear antibody test (ANA)
[not specific for sjorgen’s syndrome + -ve test makes sjogren’s syndrome unlikely] - Rheumatoid Factor test
[seen in primary and secondary sjogre’s syndrome] - Anti-SS-A (Ro)
[abnormal in Sjögren’s syndrome + associated with neonatal lupus] - Anti-SS-B (La)
[abnormal in Sjogren’s syndrome + associated with neonatal lupus]
What are the other ways you can diagnose Sjogren’s syndrome?
- Schirmer test
- Salivary test
[salivary gland scintigraphy + whole sialometry]
What is Salivary testing?
- Salivary gland scintigraphy
[a nuclear medicine test where you administer a radiotracer + look at uptake of radiotracer by salivary glands -> if SG destroyed by immune process -> low uptake of radiotracer] - Whole sialometry
[measurement of saliva production -> Patient collects all saliva over 15 mins -> sample weighed]
What is the Schirmer test and how is it conducted?
A test used in the diagnosis of Sjögren’s syndrome that tests the reflex of tear production
1) Filter paper placed near lower eyelid
2) Patient closes eyes
3) Amount of wetting (mm) measured over 5 mins
How can you make the diagnosis of Sjögren’s syndrome?
If you have any 4 of 6 criteria + must include either histopathology or autoantibodies
- Eye symptoms
- Oral symptoms
- Ocular signs (Schirmer test)
- Oral signs (Salivary testing)
- Biopsy: Lymphocytic sialadenitis
- Anti-SSa or Anti-SSb
What is the treatment of Sjögren’s syndrome?
- Good oral hygiene
- Artificial saliva
- “Pilocarpine” (A muscarinic agonist)
[stimulates acetylcholine muscarinic receptors to increase flow of fluid from salivary glands + lacrimal duct] - Steroids
[for extra-glandular manifestations of the disease]
What are the complications of Sjögren’s syndrome?
- B cell lymphoma
[presents as a persistent unilateral swollen gland which may mimic past swelling] - Neonatal lupus
[occurs when maternal antibodies cross the placenta and affect the foetus
What are the risks for Neonatal lupus?
- Mothers with Systemic lupus erythematosus and/or Sjögren’s syndrome
- +SSA/Ro or +SSB/La antibodies
What are the symptoms for Neonatal lupus?
Symptoms of Neonatal lupus occur at birth or during the first few weeks of life
SYMPTOMS:
- Rash
[multiple red, circular lesions on face + scalp]
- Congenital complete heart block
[slow heart rate (50s) + does not respond to steroids + babies usually need a lifetime pacemaker implanted]
