Sjogren's Flashcards
What is primary Sj?
Chronic autoimmune inflammatory disorder (lymphocytic) that destroys exocrine glands
Clinical hallmarks of primary Sj?
keratoconjunctivitis sicca (dry eyes)
xerostomia (dry mouth)
parotid gland swelling
Other clinical features of Sj?
fatigue Raynaud's phenomenon polyarthralgia/arthritis interstitial lung disease neuropathy purpura
What is secondary Sj?
complication of another autoimmune CT dz,
RA and SLE common
Epidemiology?
90% F
middle age*
How do you test eyes for Sj?
Schimer’s Test: filter paper documents decreased tear flow
note: eyes = dry, burning, photosensivity, etc
Xerostomia results in…
- fissuring/ulceration of lips, tongue, buccal membranes
2. difficulty chewing/swallowing
What glands may enlarge?
Parotid and/or submandibular salivary gland
*often unilateral
Other exocrine gland involvement?
Skin, vaginal, biliary tree inflammation-cirrhosis, chronic atrophic gastritis
Associated malignancy?
NHL
Renal involvement associated with Sj?
Renal tubular defects
*result in nephrogenic diabetes insipidus or renal tubular acidosis
Lung involvement associated with Sj?
- Pulmonary fibrosis
- lymphoid interstitial lung disease
* can progress to lymphoma
Neuromuscular syndromes associated with Sj?
myositis
peripheral or cranial neuropathies
seizures
encephalopathy
Obstetrics conditions associated with Sj?
recurrent fetal loss (anti-PL)
Neonatal lupus
small for gest age
Histology?
infiltration of tissues by lymphocytes and plasma cells with loss of secretory epithelium in exocrine glands
Immunologic Features?
AutoAbs
Monoglonal gammopathy
Cyroglobulinemia
Auto abs?
ANA
High titers of RF
Anti-Ro/SSa
Ant-La/SSB
Pathogenesis:
What is the result of T cell activation?
- -production of pro-inflmm cytokines (IL1, IL6, TNF)
- -secretion of protease
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
Pathogenesis:
How is Ach decreased in Sj?
- release of Ach inhibited by cytokines
2. upregulated production of AchE = increased breakdown of Ach in the epilemmal space
Pathogenesis:
What channels or receptors are altered in Sj?
- blockade of M3R by autoantibodies
- altered expression of aquaporin 5 (AQP5)
(water movement through apical cell mem)
