Sjogren's Syndrome Flashcards
Sjogren’s Syndrome and PBC - Example Question
A 54 year old lady with known Sjogren’s syndrome presents to her GP with lethargy. There are no abnormal findings on physical examination. The patient takes no regular medication apart from artificial tears. She drinks 10 units of alcohol a week and doesn’t smoke. Her GP refers her to your clinic as her routine bloods come back showing the following:
Hb 140 g/l Na+ 134 mmol/l Bilirubin 6 µmol/l Platelets 225 * 109/l K+ 3.7 mmol/l ALP 650 u/l WBC 6.7 * 109/l Urea 4.2 mmol/l ALT 36 u/l Neuts 4 * 109/l Creatinine 90 µmol/l
What is the most likely diagnosis?
Gall stones Primary sclerosing cholangitis Sarcoma Paget's disease > Primary biliary cirrhosis
Sjogren’s syndrome is associated with primary biliary cirrhosis (PBC). All the options could lead to a raised alkaline phosphatase but PBC has the greatest association with Sjogren’s syndrome.
Sjogren’s Syndrome is associated with which malignancy?
Lymphoid malignancies eg Lymphoma
Sjogren’s Syndrome Antibodies
RF +ve = 100%!
ANA +ve = 70%
anti-Ro +ve = 70%
anti-La +ve = 30%
Sjogren’s Syndrome
= autoimmune disorder affecting exocrine glands resulting in DRY mucosal surfaces. May be primary (PSS) or 2dry due to RA or other CTDs where it usually develops 10 years after initial onset.
Much more common in F (ratio 9:1)
Marked increased risk of lymphoid malignancy (40-60 fold!)
Sjogren’s Syndrome - Fx
- dry eyes: Keratoconjunctivitis Sicca
- dry mouth
- vaginal dryness
- arthralgia
- Raynaud’s
- myalgia
- sensory polyneuropathy
- renal tubular acidosis (usually subclinical)
Sjogren’s Syndrome - Histology
Focal lymphocyte infiltration
Sjogren’s Syndrome - Schirmer’s test
Filter paper near conjunctival sac to measure tear formation
Sjogren’s Syndrome Mx
- Artificial saliva and tears
- Pilocarpine may stimulate saliva production
