Sjogren Syndrome

Question 1

epi

Answer 1

30-50 yo, females, 1/3 with extraglandular disease, a/w other MCTD

Question 2

what are the common antibodies

Answer 2

anti-fodrin, anti-SSA/Ro, and less likely anti-SSB/La

Question 3

what kind of cancer do we see in these patients?

Answer 3

B cell lymphoma (**MALT lymphoma and non-hodgkins lymphoma, TNFAIP3 germline abnormality)

**felty syndrome - also a/w lymphomas and leukemias

Question 4

signs/symptoms

Answer 4

(1) xerophthalmia - dry eye, lacrimal gland
(2) xerostomia - dry mouth, affects parotid/submandibular gland
(3) Vaginal xerosis

Extranglandular findings:

(4) xerosis **
(5) vasculitis **
(6) systemic findings -> neuro, MSK, kidney (glomerulonephritis)

OFTEN #1 and 2 require > 50% of glandular destruction so will see later in disease process, while #4 and 5 are more common

Question 5

ACR diagnostic criteria

Answer 5

At least 2 of the following:

• anti SSA/Ro or antiSSB/La OR +RF and ANA > 1:320
• positive labial salivary gland bx
• xerophthalmia with ocular staining score > 3

Question 6

who has poor prognosis

Answer 6

patients with extranglandular findings

Question 7

treatment options

Answer 7

xerophthalmia -> eye drops, punctae occlusion, cyclosporine drops

xerostomia -> artificial saliva, frequent water intake, sugar free gum, pilocarpine/cevimeline, good dental hygiene, and avoid ETOH/smoking/soda