Autoimmune Disease Flashcards
Mutation or knockout of which gene causes IPEX disease and what are the resulting effects?
9Foxp3
decreased function of Treg cells, CD4 and CD25
what inhibitory cytokines do Treg cells release
IL-10 IL-35 and TFGb
aside from releasing inhibitory cytokines, what other funtions do Treg cells have?
they inhibit effector T cells, release Granzymes against apoptotic effector cells, target DCs to inhibit their maturation and function
which organ does Pemphigas vulgaris effect
skin
what drugs are used to treat Pemphigus Vulgaris
corticosteroids and immunosupressive drugs such as rituximab
what are the initial effects of pemphigus vulgaris
erosion of the mucosa- loss of epithelial cell adhesion causing detachment of cells. IgG and complement target desmosomes (structures required for cell adhesion)
which class of hypersensitivity reactions does pemphigus vulgaris belong to
type 2 hypersensitivity
which proteins are targeted by antibodies in pemphigus vulgaris
Dsg1 and Dsg3
what are the symptoms of pemphigus vulgaris
blistering of the epidermis
in which country is the edemic form of pemphigus foliacious found
brazil
what are antibodies produced against in the autoimmune disease Lupus erythematosus
nuclear antigens
what are the triggers for chronic discoid Lupus erythematosus
Uv light, infection, cold, stress
what are the symptoms of neonatal LE
skin rash, congenital atrioventricular block, thrombocytopenia, leukopenia and anaemia
how do neonates develop LE
acquire it transplacentally
why is rapid and efficient clearance of apoptotic debris key to preventing autoimmune disease
as failing this autoantigens become accesible to the immune system resulting in an immune response