Pathology: autoimmunity Flashcards
B7.1 & B7.2
co-stimulatory molecules for T cells on APC
up regulated during infection
CD28
T cell
binds B7 to activate T cell
CTLA-4
cytotoxic T-lymphocyte-associated protein 4
binds to B7 more strongly than CD28
prevents B7 from activating T cell
ipilimumab
Ab that blocks CTLA-4
treat tumors that use CTLA-4 to avoid immune system
PD-1
programmed death-1
binds B7 and prevents activation of T cell
some tumors and viruses
pembrolizumab
Ab that blocks PD-1
nivolumab
Ab that blocks PD-1
anti-dsDNA
autoantibody in SLE
formation of immune complexes
anti-Smith
autoantibody in SLE
formation of immune complexes
Anti-RNP U1
autoantibody against RNA polymerase
associated with SLE and mixed connective tissue disease
formation of immune complexes
Anti-SS-A (Ro)
autoantibody in Sjogren syndrome, SLE, and rheumatic diseases
Anti-SS-B (La)
autoantibody in Sjogren and SLE
Anti-Scl-70
autoantibody against DNA topoisomerase I
systemic sclerosis
Anti-histones
autoantibody in SLE
formation of immune complexes
Anti-centromere
autoantibody in limited scleroderma, systemic sclerosis, and CREST syndrome
Anti-phospholipid
autoantibody in antiphospholipid syndrome and SLE
thrombotic episodes
Anti-Jo1
autoantibody against histidyl tRNA ligase
inflammatory myopathies
Anti-mitochondrial
autoantibody in primary biliary cirrhosis
Anti-neutrophil cytoplasmic
autoantibody in various vasculitides
Anti-eTg
autoantibody in herpetiformis
ANA
autoantibody against nuclear components
associated with SLE
deficiency of C1q, C2 or C4 can impair clearance of apoptotic cells and nucleic acid fragments predisposes to SLE
HLA-B27
predisposition for ankylosing spondylitis
ankylosing spondylitis
destroys articular cartilage of sacroiliac joins and apophyseal joints between spinal tuberosities and processes: bony fusion of joints
Sym: lower back pain and spinal stiffness in middle age
PTPN22 polymorphisms
tyrosine phosphatase
excessive activation of lymphocytes
associated with RA and T1DM
Rheumatoid arthritis
autoimmune attack on small joints of hands and feet, especially MCP and MTP
CD25
IL-2 receptor
polymorphisms in alpha chain of IL-2 and IL-7 receptor
associated with Multiple Sclerosis
dysfunction of Treg cells
multiple sclerosis
autoimmune attack on myelin
neurological symptoms
anti-CCP
autoantibody agains cyclic citrullinated peptide
associated with RA
anti-U3 RNP
autoantibody against RNA polymerase
associated with systemic sclerosis
anti-myleoperoxidase
perinuclear anti-neutrophil cytoplasmic (P-ANCA)
associated with microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg Strauss)
anti-proteinase-3
diffuse cytoplasmic anti-neutrophil cytoplasmic (C-ANCA)
associated with granulomatosis with polyangiitis (Wegner’s)
cyclosporine
calcineurin inhibitor
nephrotoxicity can be confused with kidney rejection: isometric vacuolization of tubular epithelial cell cytoplasm, tubular calcifications, giant mitochondria
tacrolimus
binds FK506
calcineurin inhibitor
MOA calcineurin inhibitors
bind immunophilins that then inhibit the phosphatase calcneurin
calcneurin promotes secretion of TNF, IFN-gamma, IL-2, and IL-4
mycophenolate nofetil
inhibits IMP dehydorgenase in guanine synthesis
inhibits lymphocyte proliferation
azathioprine
purine analogue that inhibits DNA replication
hydroxychloroquine
anti-inflammatory
TNF-alpha inhibitors
- infliximab
- etanercept
- adalimumab
Belimumab
Ab to B cell activating factor
rituximab
Ab inhibits CD20 on B cell
tocilizumab
Ab inhibits IL-6
alemtuzumab
Ab to CD52
C4d
complement C4 degradation
deposited in peri-tubular capillaries in some cases of kidney transplant rejection
BK virus
polyomavirus
in kidney transplant: intranuclear inclusion bodies and epithelial cell injury and lysis
PTLD
post-transplant lymphoproliferative disorder
can become a lymphoma
associated with EBV