Immuno path III - Autoimmune disorders continued. Flashcards
(19 cards)
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.
What is Rheumatoid factor?
Production of IgM auto-ab that binds to IgG causing type III synovitis.
What is Pannus formation and what is it related to?
Related to rheumatoid arthritis.
Accumulation of grannular tissue, synovial and inflammatory cells as well as connective tissue in the affected joint.
What is seen more frequently in juvenile arthritis?
Systemic complications including pericarditis, myocarditis, pulmonary fibrosis and GN.
NO RF SEEN IN THES PTS.
Clinical remission can occur in up to 50% of cases.
What is the ANA ab associated with mixed connective tissue disease?
Ribonucleoprotein (Anti-U1 RNP)
