Immuno path III - Autoimmune disorders continued. Flashcards
This autoimmune disorder is characterized by the destruction of exocrine glands with the ocassional affect on GI, GU, and Respiratory tracts.
Sjorgens syndrome
Occurring 90% in females.
What is Sicca syndrome and how common is it?
This is Sjorgens syndrome causing damage only to the lacrimal and salivary glands.
Occurs in 40% of Sjorgens patients.
What is a possible mechanism for the cause of Sjorgens syndrome?
Infection by SuperAg may trigger CD4 cells to react against glandular epithelial cells.
ANAs to SS-A (RO) and SS-B (La) are indicitive of what disease?
90% of the time are related to Sjorgens syndrome.
Sjorgens patients are at 40% increased risk of what cancer?
B-cell lymphoma possibly due to development of neoplastic, monoclonal cell line via polyclonal B cell activation.
Which ANA is related to causing systemic Sjorgens syndrome ?
Ro AKA SS-A Ab.
What causes the pathogenesis of systemic sclerosis?
B-cells become activated for unknown reason which then activate T cells to produce cytokines that promote fibrogenesis and excessive collagen in the skin and viscera.
B cells in systemic sclerosis produce which ANAs?
anti-DNA topoisomerase I (anti-Scl-70) ab.
The limited variant of SS is positive to which Ab in 90% of cases?
Positive to anticentromere Ab!
Leads to limited involvement and late, slow progressing visceral involvement.
What does CREST syndrome relate to and what is it a variant of?
C - Calcinosis R - Reynauds E - Esophageal dysmotility S - Sclerodactly T - Telangiectasia A limited variant of Systemic Sclerosis.
Esophageal atrophy and collagenization is a clinical sign of what defect?
CREST syndrome, the limited form of Systemic Sclerosis.
Cor pulmonale of the heart is a clinical manifestation of what systemic disorder?
Systemic Sclerosis occuring in >50% of patients.
What is the 10 year survival rate for Systemic Sclerosis?
35-70%
COD:
Renal failure, cardiac failure, pulmonary insuffciency, GI malabsorption.
What is the male to female ratio of infection by RA?
2-3F:1M within ages 40-70.
The pathogenesis of rheumathoid arthritis is as follows:
Activation of CD4 T cells leading to production of damaging cytokines and proliferation of synovial cells and fibroblasts.
TNF leads to leukocyte recruitment.
