Immunology Flashcards
top 6 most common AI conditions in order most to least common
Graves > RA > Hashimotos thyroiditis > vitiligo
>T1D > pernicious anaemia
IPEX syndrome - mono/polygenic AI?
stands for
age =
monogenic
Immune dysregulation, Polyendocrinopathy, Enteropathy, X linked inheritance
early childhood
symptoms of IPEX syndrome
severe infections
intractable diarrhoea
eczema
early onset T1D
pathogenesis of IPEX syndrome = \_\_\_ gene (for regulation of T cell dev) > failure of peripheral \_\_\_ (inactivation of \_\_\_ that escape central \_\_\_ (removal of \_\_\_\_ in primary lymphoid tissues)
FOXP3 tolerance self-reactive lymphocytes tolerance self-reactive lymphocytes
treatment of IPEX syndrome
HSCT (haemopoietic stem cell transplant) cures it
IS and TPN
broad classes of genes involved in polygenic AI (3)
HLA
sex
immune regulatory
HLA ass with ank spond
B27 (90% relative risk)
HLA ass with RA
DR4 (10%)
HLA ass with SLE
DR3 (5%)
HLA ass with MS
DR2 (5%)
HLA ass with T1D
DR4/3 (20%)
HLA ass with myasthenia gravis
DR3 (10%)
in myasthenia gravis HLA DR3 is predisposing for ___ and protective of __ onset
predisp - early onset
protect - late onset
molecular mimicry =
in infection eg Strep the pathogens antigens cause cross-reactive Igs to be produced that attack self
eg. Rheumatic fever
SLE is a type __ H/S disease
3