Flashcards in Chp. 2 Sjogren Syndrome Deck (11):
What is Sjogren Syndrome?
Autoimmune destruction of lacrimal and salivary glands.
Can be a primary disease (sicca syndrome) or associated with another autoimmune disease, ESPECIALLY RA
What cell mediates the destruction to the lacrimal and salivary glands? What kind of HSR is this?
Lymphocyte mediated damage (look for lymphocytic infiltrates)
Type 4 HSR
How does it classically present?
Dry eyes (keratoconjuctivitis sicca) dry mouth (xerostomia) and constant cavities in old women.
Cant chew a cracker, dirt in my eyes
What could the dry eyes and and dry mouth lead to (besides cavities)
Ulceration of corneal epithelium and oral mucosa
What other autoimmune disease is Sjogren's usually associated with? Even if RA isn't there, what could be present?
Rheumatoid Factor (if this is present, but no RA, then this is primary Sjogren's)
What Abs are present in these patients?
ANA (anti-nuclear Ab)
Anti-ribonucleoprotein Abs which are
-Anti-SSA/Ro and Anti SSB/La
However, if someone has an Anti-SSA or B, it DOES NOT NEC MEAN THEY HAVE SJOGRENS
What are the anti-SSA and anti-SSb associated with?
Extraglandular manifestations (neuropathy, joint issues)
In addition, a subset of lupus patients have these Abs, SO SCREEN PREGGOS!!
Preggos with which anti Ab are at risk for what complications?
Risk for Neonatal Lupus and Congenital Heart Block
What is an additional criterion for Dx of Sjogren?
Lymphocytic Sialadenitis upon a lip biopsy. This shows lymphocytes actually destroying the glands.
This could exclude other causes such as sarcoidosis, amyloidosis, drug side effects
What are Sjogren's patients at increased risk for?
Increased risk for B cell (marginal zone) lymphoma which presents as unilateral enlargement of parotid gland late in disease
Please recognize this is a MALToma!!
This is like the 15-20 yr Sjogren's pt, so late in the course of the disease