Flashcards in Sjogren's Deck (21):
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?
interstitial lung disease
What is secondary Sj?
complication of another autoimmune CT dz,
(RA and SLE common)
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...
1. fissuring/ulceration of lips, tongue, buccal membranes
2. difficulty chewing/swallowing
What glands may enlarge?
Parotid and/or submandibular salivary gland
Other exocrine gland involvement?
Skin, vaginal, biliary tree inflammation-cirrhosis, chronic atrophic gastritis
Renal involvement associated with Sj?
Renal tubular defects
*result in nephrogenic diabetes insipidus or renal tubular acidosis
Lung involvement associated with Sj?
1. Pulmonary fibrosis
2. lymphoid interstitial lung disease
*can progress to lymphoma
Neuromuscular syndromes associated with Sj?
peripheral or cranial neuropathies
Obstetrics conditions associated with Sj?
recurrent fetal loss (anti-PL)
small for gest age
infiltration of tissues by lymphocytes and plasma cells with loss of secretory epithelium in exocrine glands
High titers of RF
What is the result of T cell activation?
--production of pro-inflmm cytokines (IL1, IL6, TNF)
--secretion of protease
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
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