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Flashcards in Sjogren's Deck (21)
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1
Q

What is primary Sj?

A

Chronic autoimmune inflammatory disorder (lymphocytic) that destroys exocrine glands

2
Q

Clinical hallmarks of primary Sj?

A

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

3
Q

Other clinical features of Sj?

A
fatigue
Raynaud's phenomenon
polyarthralgia/arthritis
interstitial lung disease
neuropathy
purpura
4
Q

What is secondary Sj?

A

complication of another autoimmune CT dz,

RA and SLE common

5
Q

Epidemiology?

A

90% F

middle age*

6
Q

How do you test eyes for Sj?

A

Schimer’s Test: filter paper documents decreased tear flow

note: eyes = dry, burning, photosensivity, etc

7
Q

Xerostomia results in…

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

2. difficulty chewing/swallowing

8
Q

What glands may enlarge?

A

Parotid and/or submandibular salivary gland

*often unilateral

9
Q

Other exocrine gland involvement?

A

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

10
Q

Associated malignancy?

A

NHL

11
Q

Renal involvement associated with Sj?

A

Renal tubular defects

*result in nephrogenic diabetes insipidus or renal tubular acidosis

12
Q

Lung involvement associated with Sj?

A
  1. Pulmonary fibrosis
  2. lymphoid interstitial lung disease
    * can progress to lymphoma
13
Q

Neuromuscular syndromes associated with Sj?

A

myositis
peripheral or cranial neuropathies
seizures
encephalopathy

14
Q

Obstetrics conditions associated with Sj?

A

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

15
Q

Histology?

A

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

16
Q

Immunologic Features?

A

AutoAbs
Monoglonal gammopathy
Cyroglobulinemia

17
Q

Auto abs?

A

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

18
Q

Pathogenesis:

What is the result of T cell activation?

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

- -secretion of protease

19
Q

Pathogenesis:

How do epithelial cells play a role?

A

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

*cell surface molecules involved in activation of apoptotic pathways

20
Q

Pathogenesis:

How is Ach decreased in Sj?

A
  1. release of Ach inhibited by cytokines

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

21
Q

Pathogenesis:

What channels or receptors are altered in Sj?

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