MDTI - Rheumatology 10 Flash Cards

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Title: MDTI - Rheumatology 10
Description: Sjogren's
Number of Cards: 20
Save Count: 0
Author: kgar18
Created: 2012-02-05
Tags: sjogren's
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    • Question
    • Answer
    • Side 3
    • What is Sjogrens syndrome?
    • 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.
    • What are the signs/symptoms of Sjogrens?
    • Dry eyes, mouth, nose, skin, etc

      Musculoskeletal pain

      Fatigue, malaise
    • What are the 2 types of sjogrens?
    • Primary: Sicca symptoms develop in healthy person

      Secondary: Sicca symptoms develope in a person with a pre-existing connective tissue disease (ie RA)
    • What are the 3 stages of Sjogrens:
    • 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)
    • What is the pathogenesis of Sjogrens?
    • 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
    • serous acinar cell produce what?

      mucous acinar cells produce what?
    • Serous: thin watery saliva

      Mucous: thicker saliva rich in mucins to lubricate food
    • What is a focus?
    • A concentration (in sjorgrens) in a lymphepithelia lesion of 50 or more mononuclear cells
    • What does an early stage sjogrens salivary gland biopsy show?
    • Lymphoepithelial Lesion

      Clusters of lymphocytes around epithelium

      - See dialated ducts
    • What chemokine is overexpressed in Sjogrens?

      What does it do?
    • CXCL12

      Attracts T cells and dendritic cells
    • What new chemokine is expressed by salivary gland epithelial cells (SGECs) in sjorgrens?

      What does it do?
    • CXCL13

      Attracts B cell
    • What type of cells do SGEC's mimic in Sjorgrens?
    • APC's

      Express MCH I & II
    • What is a focus score?
    • The number of foci seen in a glandular biopsy.

      >1 per 4mm2 give a positive biopsy
    • What is the female:male ratio in sjorgrens?
    • 9:1
    • What are 3 (general) possible causes of sjogrens?
    • 1. Genetics

      2. Hormonal Factors

      3. Environmental agents (viruses?)
    • What "immune system" is activated in Sjogrens?
    • Type I interferon system

      Include IFN-a (most important cytokine)
    • What are the 2 main causes of dryness in sjogrens?
    • 1. Parenchymal loss (apoptosis)

      2. Non-apoptotic mechanisms are also likely
      - SS patients often show large amount of normal appearing acinar tissues in biopsies
    • What are the 2 proposed causes for the non-apoptotic glandular hypofunction in SSs?
    • 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)
    • What severe condition can SS progress to?
    • Lymphoma - usually a marginal zone lymphoma (MALT subtype)
    • What B cell activator is seen in SS?
    • BAFF (B-cell activation factor)

      AKA: Blys

      RECALL: Also seen in SLE
    • What are the effects of BAFF?
    • B cell hyperactivty

      B cell autoimmunity

      Extranodal germinal center creation

      Ultimately can cause lymphoma