Immunopathology III Flashcards
(30 cards)
What is Sjorgrens syndrome marked by?
Destruction of exocrine glands in the body
i.e.: Lacrimal and salivary glands.
What sex does sjorgens disease most commonly affect?
90% of cases are wtihin women.
What is sicca syndrome?
A type of sjorgens syndrome in which there is a decrease in tears and saliva.
What is the pathogenesis for sjorgens syndrome?
CD4 T cells kill self Ag in glands.
Systemic B cell hyperactivity occurs with 75% of cases positive for RF
Which specific ANAs does sjorgens syndrome have?
Specific ANAs to ribonucleoproteins
SS-A (Ro)
and
SS-B (La) - Very common.
Systemic Sjorgens syndrome is associated with what ANA?
SS-A ab
Ro
What is the male to female ratio for systemic sclerosis?
3:1 F:M
typically occurring around 50-60 years.
What cytokine causes mass activation of fibroblasts in Systemic Sclerosis?
TGF-B
What is the limited varient of systemic sclerosis?
CREST syndrome with late visceral involvement in a rather benign course and limited skin involvmenet.
Look for anti-centromere ab.
What is the major characteristic test for CREST syndrome identification?
Anti centromere antibody
What does CREST stand for?
Calcinosis - Calcium deposits in skin
Raynauds phenomenon
Esophageal dysfunction
Sclerodactyly (Thickening and tightening of skin on hands)
Telangiectasis (Dilation of capillaries on skin)
What is the diffuse variant of CREST?
Widespread skin involvement at onset with rapid progression due to Ab to DNA topoisomerase I (Anti-ScL-70)
What is the reactive antibodies produced to the diffuse variant of CREST?
Typically Ab to DNA topoisomerase I (anti-Scl-70) occuring in 70% of cases.
Which disease ca cause vascular obliteration and fibrosis within the esophagus?
CREST
Cor pulmonale can be caused by what?
Systemic Sclerosis within the lungs occuring >50% of the time.
What is the ten year survival rate for Systemic sclerosis?
Varies from 35-70% depending on which organs are involved.
What are the typical organ problems that result in death caused by systemic sclerosis?
Renal
Cardiac
Pulmonary
GI dysfunction or failure.
What genetic issue leads to RA?
HLA-DRB1 and PTPN22 gene for tryrosine phosphatase begins the synovitis that is then continued by CD4 T cells making cytokines.
What exactly is rheumatoid factor?
An auto antibody IgM that has an Fc portion autologous to IgG in 80% of cases.
Citrullinated peptides contribute to what disease state?
Rheumatoid arthritis.
What is pannus formation and what is it linked to?
Granulation tissue, synovial and inflammatory cells with fibrous conective tissue.
Linked to RA.
In 25% of RA cases, rheumatoid nodules will appear at whcih locations?
Appear at skin pressure points such as the elbow or occiput.
Also appear in the viscera of the lungs, spleen, and heart.
In cases of severe rheumatoid arthritis what can occur in the blood vessels??
Small vessel vasculitis with high tighters of RF
How does RF damage the body?
RF binds with IgG and forms antibody antigen complexes causing type III damage to small vessels in severe cases of RA.
