Sjogren's Flashcards Preview

MS/Rheum > Sjogren's > Flashcards

Flashcards in Sjogren's Deck (21):
1

What is primary Sj?

Chronic autoimmune inflammatory disorder (lymphocytic) that destroys exocrine glands

2

Clinical hallmarks of primary Sj?

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

3

Other clinical features of Sj?

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

4

What is secondary Sj?

complication of another autoimmune CT dz,
(RA and SLE common)

5

Epidemiology?

90% F
middle age*

6

How do you test eyes for Sj?

Schimer’s Test: filter paper documents decreased tear flow

(note: eyes = dry, burning, photosensivity, etc)

7

Xerostomia results in...

1. fissuring/ulceration of lips, tongue, buccal membranes
2. difficulty chewing/swallowing

8

What glands may enlarge?

Parotid and/or submandibular salivary gland

*often unilateral

9

Other exocrine gland involvement?

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

10

Associated malignancy?

NHL

11

Renal involvement associated with Sj?

Renal tubular defects

*result in nephrogenic diabetes insipidus or renal tubular acidosis

12

Lung involvement associated with Sj?

1. Pulmonary fibrosis

2. lymphoid interstitial lung disease
*can progress to lymphoma

13

Neuromuscular syndromes associated with Sj?

myositis
peripheral or cranial neuropathies
seizures
encephalopathy

14

Obstetrics conditions associated with Sj?

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

15

Histology?

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

16

Immunologic Features?

AutoAbs
Monoglonal gammopathy
Cyroglobulinemia

17

Auto abs?

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

18

Pathogenesis:
What is the result of T cell activation?

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

19

Pathogenesis:
How do epithelial cells play a role?

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

*cell surface molecules involved in activation of apoptotic pathways

20

Pathogenesis:
How is Ach decreased in Sj?

1. release of Ach inhibited by cytokines
2. upregulated production of AchE = increased breakdown of Ach in the epilemmal space

21

Pathogenesis:
What channels or receptors are altered in Sj?

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