Flashcards in Immunology Deck (29)
what are monogenic autoimmune disorders?
autoimmune disease caused by single gene defects
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?
what type of HLA increases the susceptibility of type 1 diabetes?
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?
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?
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?
Immunologic phenomena (ie anti-dsDNA, anti-Sm)
what type of hypersensitivity is rheumatoid arthritis?
what cell infiltrate in the synovium characterises rheumatoid arthritis?
initially- helper T cells
secondary- phagocytes, activated B cells and antibody
what antibodies can be present in rheumatoid arthritis?
what are the associated conditions with anti-CCP antibody?
what are the 4 main associated conditions with ANA? (anti-nuclear antibody)
what are associated conditions with anti-dsDNA? (anti-double stranded DNA)?
what are the associated conditions with anti-Sm?
what are the associated conditions with anti-Ro?
SLE, Sjogren's syndrome
what are the associated conditions with anti-La?
what are the associated conditions with anti-centromere?
limited systemic sclerosis
what are the associated conditions with anti-Scl-70?
diffuse systemic sclerosis
what are the associated conditions with anti-RNP?
mixed connective tissue disease
what are the associated conditions with anti-cardiolipin antibody?