Nelson Flashcards
What is the underlying pathologic mechanism of SLE?
Anti-nuclear antibodies–> immune complexes (3)
- smith and dsDNA abs are dx
- there is some type 2 as well
What does anti-phospholipid abs in SLE cause?
Thrombosis- young stroke
- increase INR
- false + syphillis test
- can also lead to miscarriages and cerebral ischemia
What is the underlying pathologic mechanism for RA?
Cyclic citrullinated proteins are dx
- infection and smoking lead to increase production of CCPs
- HLA-DRB1
What is Sjogren syndrome?
Chronic dry eyes and dry mouth
- SSA and SSB not specific but you need lip biopsy to confirm dx
- most common in middle ages women
- can lead to marginal zone lymphoma
What is the underlying pathogenesis of scleroderma?
Abs against Scl-70 (DNA topoisomerase)
- diffuse or limited
- CREST
Dermatomyosisits vs polymyositis?
Elevated CK and treat with immunosuppressants
- Derm has discoloration under eyes with orbit edema
- poly- anti-Jo1 against muscle cells
- derm actived b and t cells leads to abs with complement
What should one screen for with a pt with dermatomyositis?
Malignancy (15-25% of pts
What 2 ag’s are most important in determining the likelihood of rejection?
HLA
ABO
What is the difference between direct and indirect cellular rejection?
Direct is where the graft has the mediated response
- indirect is host APCs are responsible
How about the humoral rejection?
Ab mediated
- preformed or develop after transplantation
- prior preg, prior transplant or blood transfusion
what cause GVHD?
When a immunologically competent donor T cell recognize the recipients HLA as foreign ags
What is AL amyloid light chain?
Accumulation of either Lambda or kappa light chain produced from free immunoglobulin light chain protein by plasma cells
What is AA amyloid ass?
chronic inflammation
- SSA protein proteolysis
- SAA is an acute phase protein
What is Beta amyloid?
From amyloid precursor protein
- A dz and cerebral plaques
What is trasthyretin (TTR)?
transport thyroxine and retinal
- cause heritable nueropathic and cardiomyopathy amyloidosis
what is B2 microglobulin?
cannot be filtered via dialysis
What is the mechanism of light chain restriction?
In infectious dz we see polyclonal proliferation but in neoplasms we see monoclonol
- thus we see either kappa or lambda single ab increase in neoplasia
What does reactive folicular hyperplasia have that a malignancy doesnt?
tangible body macs
Type 1 hypersense?
IgE mediated and must have previous sensitzed to ag
- ATOPY- genetic predisposition
- late phase response
- fall in bp, bronchospasm and larnygeal edema
Type 2 hypersense?
Ab mediated
- Linear deposition
- Myasthenia gravis or good pasture syndrome
Type 3 hypersense?
Immune complex mediated
- Ab complexes deposit on endothelial wall
- kidney and synovium common site
- fibroid necrosis
- vasculitis, glomerulonephritis and arthritis
Typer 4 hypersense?
Cell mediated
- T cell mediated and no ab
- Mantoux test- delayed type (CD4)
- DM1 and graft rejection mediated by CD8
- leads to granuloma formation
- caseating have central necrosis
- non case in chrons or sarcoidosis
what are passenger mutations?
no phenotypic consequence
- occur during cancer growth but dont contribute to development of cancer themselves (driver mutations)
without angiogensis what size can cancer cells grow to?
1-2mm