Chp. 2 Sjogren Syndrome

Question 1

What is Sjogren Syndrome?

Answer 1

Autoimmune destruction of lacrimal and salivary glands.

Can be a primary disease (sicca syndrome) or associated with another autoimmune disease, ESPECIALLY RA

Question 2

What cell mediates the destruction to the lacrimal and salivary glands? What kind of HSR is this?

Answer 2

Lymphocyte mediated damage (look for lymphocytic infiltrates)

Type 4 HSR

Question 3

How does it classically present?

Answer 3

Dry eyes (keratoconjuctivitis sicca) dry mouth (xerostomia) and constant cavities in old women.

Cant chew a cracker, dirt in my eyes

Question 4

What could the dry eyes and and dry mouth lead to (besides cavities)

Answer 4

Ulceration of corneal epithelium and oral mucosa

Question 5

What other autoimmune disease is Sjogren’s usually associated with? Even if RA isn’t there, what could be present?

Answer 5

Rheumatoid Arthritis

Rheumatoid Factor (if this is present, but no RA, then this is primary Sjogren’s)

Question 6

What Abs are present in these patients?

Answer 6

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

Question 7

What are the anti-SSA and anti-SSb associated with?

Answer 7

Extraglandular manifestations (neuropathy, joint issues)

In addition, a subset of lupus patients have these Abs, SO SCREEN PREGGOS!!

Question 8

Preggos with which anti Ab are at risk for what complications?

Answer 8

Anti-SSA

Risk for Neonatal Lupus and Congenital Heart Block

Question 9

What is an additional criterion for Dx of Sjogren?

Answer 9

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

Question 10

What are Sjogren’s patients at increased risk for?

Answer 10

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

Question 11

What do you need to Dx Sjogrens?

Answer 11

2 of 3 of the following criteria:

1. Dry eyes
2. ANA & Anti-SSA or B
3. Lymphocytic Sialadenitis (esp this one)