Sjogren's

Question 1

What is primary Sj?

Answer 1

Chronic autoimmune inflammatory disorder (lymphocytic) that destroys exocrine glands

Question 2

Clinical hallmarks of primary Sj?

Answer 2

keratoconjunctivitis sicca (dry eyes)
xerostomia (dry mouth)
parotid gland swelling

Question 3

Other clinical features of Sj?

Answer 3

fatigue
Raynaud's phenomenon
polyarthralgia/arthritis
interstitial lung disease
neuropathy
purpura

Question 4

What is secondary Sj?

Answer 4

complication of another autoimmune CT dz,

RA and SLE common

Question 5

Epidemiology?

Answer 5

90% F

middle age*

Question 6

How do you test eyes for Sj?

Answer 6

Schimer’s Test: filter paper documents decreased tear flow

note: eyes = dry, burning, photosensivity, etc

Question 7

Xerostomia results in…

Answer 7

1. fissuring/ulceration of lips, tongue, buccal membranes

2. difficulty chewing/swallowing

Question 8

What glands may enlarge?

Answer 8

Parotid and/or submandibular salivary gland

*often unilateral

Question 9

Other exocrine gland involvement?

Answer 9

Skin, vaginal, biliary tree inflammation-cirrhosis, chronic atrophic gastritis

Question 10

Associated malignancy?

Answer 10

NHL

Question 11

Renal involvement associated with Sj?

Answer 11

Renal tubular defects

*result in nephrogenic diabetes insipidus or renal tubular acidosis

Question 12

Lung involvement associated with Sj?

Answer 12

1. Pulmonary fibrosis
2. lymphoid interstitial lung disease
   * can progress to lymphoma

Question 13

Neuromuscular syndromes associated with Sj?

Answer 13

myositis
peripheral or cranial neuropathies
seizures
encephalopathy

Question 14

Obstetrics conditions associated with Sj?

Answer 14

recurrent fetal loss (anti-PL)
Neonatal lupus
small for gest age

Question 15

Histology?

Answer 15

infiltration of tissues by lymphocytes and plasma cells with loss of secretory epithelium in exocrine glands

Question 16

Immunologic Features?

Answer 16

AutoAbs
Monoglonal gammopathy
Cyroglobulinemia

Question 17

Auto abs?

Answer 17

ANA
High titers of RF
Anti-Ro/SSa
Ant-La/SSB

Question 18

Pathogenesis:

What is the result of T cell activation?

Answer 18

• -production of pro-inflmm cytokines (IL1, IL6, TNF)

- -secretion of protease

Question 19

Pathogenesis:

How do epithelial cells play a role?

Answer 19

upregulate expression of Fas (FAS) and Fas ligand (FASL)

*cell surface molecules involved in activation of apoptotic pathways

Question 20

Pathogenesis:

How is Ach decreased in Sj?

Answer 20

1. release of Ach inhibited by cytokines

2. upregulated production of AchE = increased breakdown of Ach in the epilemmal space

Question 21

Pathogenesis:

What channels or receptors are altered in Sj?

Answer 21

1. blockade of M3R by autoantibodies
2. altered expression of aquaporin 5 (AQP5)
   (water movement through apical cell mem)