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20 Cards in this Set
- Front
- Back
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What is Sjogrens syndrome?
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A chronic, autoimmune inflammatory disorder characterized by LYMPHOCYTIC INFILTRATION of EXOCRINE GLANDS leading to diminished, lacrimal, salivary gland, and glandular secretion in associated with auto-antibody production in the blood.
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What are the signs/symptoms of Sjogrens?
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Dry eyes, mouth, nose, skin, etc
Musculoskeletal pain Fatigue, malaise |
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What are the 2 types of sjogrens?
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Primary: Sicca symptoms develop in healthy person
Secondary: Sicca symptoms develope in a person with a pre-existing connective tissue disease (ie RA) |
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What are the 3 stages of Sjogrens:
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Stage I: Sicca symtoms, constitutional symptoms, muscle pain
Stage II: Internal organ involvement and vasculitis Stage III: Non-Hodkins B cell lymphomas (in salivary glands) |
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What is the pathogenesis of Sjogrens?
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Initial Phase: Injury to epithelial cells in exocrine glands and eventual apoptosis
Later Phase: - Infiltration of glands by lymphocytes and other inflammatory cells - Cytokine release - Autoantibody production - Autoimmune destruction - Decreased secretory function |
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serous acinar cell produce what?
mucous acinar cells produce what? |
Serous: thin watery saliva
Mucous: thicker saliva rich in mucins to lubricate food |
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What is a focus?
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A concentration (in sjorgrens) in a lymphepithelia lesion of 50 or more mononuclear cells
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What does an early stage sjogrens salivary gland biopsy show?
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Lymphoepithelial Lesion
Clusters of lymphocytes around epithelium - See dialated ducts |
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What chemokine is overexpressed in Sjogrens?
What does it do? |
CXCL12
Attracts T cells and dendritic cells |
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What new chemokine is expressed by salivary gland epithelial cells (SGECs) in sjorgrens?
What does it do? |
CXCL13
Attracts B cell |
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What type of cells do SGEC's mimic in Sjorgrens?
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APC's
Express MCH I & II |
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What is a focus score?
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The number of foci seen in a glandular biopsy.
>1 per 4mm2 give a positive biopsy |
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What is the female:male ratio in sjorgrens?
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9:1
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What are 3 (general) possible causes of sjogrens?
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1. Genetics
2. Hormonal Factors 3. Environmental agents (viruses?) |
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What "immune system" is activated in Sjogrens?
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Type I interferon system
Include IFN-a (most important cytokine) |
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What are the 2 main causes of dryness in sjogrens?
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1. Parenchymal loss (apoptosis)
2. Non-apoptotic mechanisms are also likely - SS patients often show large amount of normal appearing acinar tissues in biopsies |
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What are the 2 proposed causes for the non-apoptotic glandular hypofunction in SSs?
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1. Production of anti-M3R (muscarine receptor) that competitively inhibit binding of AcH
(ant-M3R antibodies found in up to 90% of patients with primary SS) 2. Increased breakdown of Ach in the epilemmel space (via elevated cholinesterase) |
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What severe condition can SS progress to?
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Lymphoma - usually a marginal zone lymphoma (MALT subtype)
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What B cell activator is seen in SS?
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BAFF (B-cell activation factor)
AKA: Blys RECALL: Also seen in SLE |
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What are the effects of BAFF?
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B cell hyperactivty
B cell autoimmunity Extranodal germinal center creation Ultimately can cause lymphoma |
