Immunology Flashcards
What does IRAK 4 deficiency lead to?
Recurrent pyogenic infections due to down regulation of the NF Kappa B signalling from TLR.
Distal to Toll Like Receptor 4/7/8/9
Where are the different types of Pattern Recognition Receptors of the innate immune system found?
Toll Like Receptors (membrane bound and intracellular) Inflammasomes (cytosol) C-type lectin receptors Nod Like receptors (Intracellular) Rig like receptors (Intracellular)
U1RNP association
Mixed connective tissue disorder
ANA negative Ribo P positive
Lupus
Often quoted as being associated with cerebral lupus/neuropsychiatric lupus
RNA polymerase 3 association
Impending scleroderma renal crisis
RNA polymerase antibodies detectable in Scleroderma
RNAP III (worse prognosis) or CEN-P or or ScL-70
Mutually exclusive
IL-4
Atopy
Exczema
Asthma
IL-12
Inflammatory
IL-10
Anti-inflammatory
Interferon Gamma
Th1/CD4 mediated
- Interferon gamma (drives B cells)
Regulatory t cells (CD4+/CD25+)
Govern peripheral tolerance
SMA (Smooth muscle antibody) associated with
Autoimmune hepatitis
Weak SMA in other liver disorders ie
Biomarker for autoimmune hepatitis activity
IgG (often elevated)
Transaminases
Autoimmune hepatitis types
Type 1: SMA (adult, often do better)
Type 2: ALKM (children, often do worse)
Lymphoplasmacytic infiltrate around the triad (interface)
Marker of PBC
Anti-mitochondrial antibody
Treatment for AIH
Induction: Steroids
Continuation: Azathioprine if required
Treatment for PBC
Urso
Anti-phospholipid syndrome risk factors for developing new thrombotic events
Recent discontinuation of anti-coagulation (particularly if previous clot as vein damaged)
Prothrombotic rank of antibodies: Cardiolipin –> Beta2 –> Lupus anticoagulant
All three = greatest risk