Immunology Flashcards
what are monogenic autoimmune disorders?
autoimmune disease caused by single gene defects
rare
What is IPEX syndrome?
Immune-dysfunction, Polyendocrinopathy, Enteropathy and X-linked inheritance syndrome:
x-linked, autoimmune, recessive disorder that presents early in childhood
what symptoms do children with IPEX present with?
type 1 diabetes (
what is the pathogenesis of IPEX syndrome?
mutation in a gene essential for development of regulatory T cells
what type of HLA increases the susceptibility of ankylosing spondylitis?
HLA B27
what type of HLA increases the susceptibility of type 1 diabetes?
HLA DR3/DR4
what class of HLA do all nucleated cells express?
HLA class I
what type of cells do HLA class I control?
cytotoxic T cells
what class of HLA do specialised antigen-presenting cells express?
HLA class II
what type of hypersensitivity is lupus?
type III
what is the fundamental abnormality in SLE?
disturbed regulation of B cell activity
why might there by very quiet heart sounds in a patient with SLE?
due to pericardial effusion
what antibody is specific for limited systemic scleroderma?
anti-centromere antibody
what complement levels would you expect to find in a patient with SLE?
low complement levels
because you have used up the store for activation
what is the criteria classification of SLE?
Serositis Oral ulcers Arthritis (non-erosive) Photosensitivity Blood disorders Renal involvement ANA Immunologic phenomena (ie anti-dsDNA, anti-Sm) Neurological disorders Malar rash Discoid rash
