immunological investigation of multisystem A.I diseases Flashcards
autoimmunity
predisposing factors
breakdown of immunological tolerance
pathological autorecognition
inflammation & tissue damage
test purpose
disease -diagnosis - subclassification - prognosis -monitering treatment -planning -selection -triage - M&E
immunology test
antinuclear antibodies antineutrophil cytoplasmic antibodies antiphospholipid antibodies complement cryoglobulins
antibody production
Th2-> Bcell
Antinuclear antibody
SLE (95%) drug induced Lupus Mixed connective tissue disorder RA sjogren scleroderma chronic inflammation old age neoplasia chronic active hepatitis
ANA
high sensitivity low specificity SLE high negative value low positive predictive value ANA negative SLE unlikely
types
homogeneous
speckled
nucleolar
peripheral
homogeneous
dsDNA -SLE (60-90%) -some autoimmune liver disease ssDNA -non specific -many inflam disorders Histone proteins -drug induced lupus
Speckled
Ro- Sjogren La- Sjogren Sm- SLE RNP- MCTD Scl-70- Scleroderma Jo-1- Polymyositis Centromere
Nucleolar
nucleolus specific RNA
- Scleroderma, systemic sclerosis & ovrlap syndromes
- clinical variants
peripheral
dsDNA
-SLE
cascade testing
screening test-> specific test
auto Ab
Anti- dsDNA= nephritis, vasculitis -Sm= nephritis, cerebral -RNP= Arthritis, myositis, Raynauds -Ro= photosensitivity, sjogren's cutaneous form of lupus
Antinuclear cytoplasmic antibodies
small vessel vasculitis
1) ANCA-associated
2) immune complex associated
ANCA
C-ANCA major autoantigen associated - proteinase 3 -> GPA P- ANCA Major autoantigen associated- Myeloperoxidase -> MPA ->EGPA (eosinophilic granulomatous polyangiitis)
ANCA
variably correlate with disease should not change solely on this for treatment not disease specific unlike most -infection -inflammation - drugs - connective tissue disorders -inflammatory BD
Antiphospholipid syndrome
Primary secondary - CT disorders - Chronic infection - Drugs ( phenytoin) Lymphoproliferative Features: Vascular thrombosis Recurrent fetal loss Livedo reticularis- small thrombi forming in post cap venule Thrombocytopenia
antiphospholipid antibodies
Anticardiolipin antibodies
Anti- B2 glycoprotein I antibodies
Lupus anticoagulant
Complement
Phagocyte chemotaxis
opsonisation
lysis of micro organisms
maintaining solubility of immune complexes
completement assays
diagnosis/ monitoring disease
detection of hereditary deficiencies
Immune complex diseaseC
C1,4,2
Recurrent neisserial infection
C 5678
Recurrent bacterial infection
C3
Cryoglobulin
immunoglobuin which reversibly precipitate out of solution at temperatures <37 degrees
types
monocloncal (plasma cell dyscrasias)
Polyclonal IgG
with monoclonal IgM (hepatitis)
Poly Clonal (CT disease)
Cryoglobulins
livedo reticularis
raynauds
peripheral cyanosis
purpura
glomerulonephritis
neuropathy
arthralgia