Immunopathology II Flashcards
Tolerance
incapability to mount an immune response against specific antigen
self tolerance
lack of responsiveness to self antigens
Central tolerance
referes to deletion of self-reactive B and T cells during their maturation inthe bone marrow and thymus
Peripheral tolerance
mechanism to eliminate self reactive T cells that escaped intrathymic delection (- selection)
- anergy
- suppression by reg. t cells
- deletion by induced apoptosis
- antigen sequestration
Anergy
T cell rendered useless after bad enconter
T cell requires two signals: MHCII and B7 (APCs)
- secondary signal B7–>CD28 is absent
- instead CTLA4 binds B7–>thus anergy
Suppression by regulatory T cells (T-regs)
- T-regs develop in the thymus and express CD4 and CD25
- mutation in Fox3, required for their dev and fx results in severe autoimmune
Deletion by Induced apoptosis
involves fas ligand system
mutaions can lead to autoimmune lyphoproliferative syndrome
antigen sequestration
some antigens are hidden from immune system due to lack of communication with blood and lymph (immune-privileged sites such as testis, eye, brain
Systemic Lupus Ertyhematosis (SLE)
unknown cause, but fundamental defect is failure to maintain self-tolerance
-characterized by presence of antinuclear antibodies
Criteria for diagnosis
Antinuclear antibodies (ANA)
Antinuclear Antibodies and Autoimmune Disease
Drug induced Lupus will have antihistones.
Antiphospholid antibody (lupus anticoagulat) is associated with?
thrombosis and recurrent miscarriages
Early complement deficiency (C3, C4, C1q) in SLE will?
impair removal by MO and favor tissue deposition
approx 5-10% patients
How are vesceral lesions mediated in SLE?
Hypersensitivity type III
What happens when cel nuclei are exposed to ANA in SLE?
cell damage occurs. those cells lose their chromatin pattern and become homogenous (hematoxylin bodies)
What does this figure represent?
Hematoxylin Bodies (SLE)
What does this figure represent?
Immune-complex deposition (Type III HS)
SLE