autoimmune disorders Flashcards
SLE
immune complexes of self antigens - deposit
complement deficiency
SLE HLA association
DR2, DR3
SLE
deficiency in complement pathway proteins - Cq1, C2, C4
SLE predisposition
uv rays, cigarettes, hormones, drugs (procainamide, hydralazine)
procainamide
SLE
hydralazine
SLE
TLR 9
recognition of self DNA in SLE
TLR-7
recognition of self RNA in SLE
SLE abnormalities
plasmacytoid DC - INF alpha
B cells - activation by nuclear ag
Failure of tolerance
SLE antibodies
antinuclear
antiphospholipids
antibodies against blood substances
SLE hypersensitivity
type iii
organs affected
vessels (fibrinoid necrosis), kidneys (glomerulonephritis), skin, joints, spleen (onion skin lesion), heart (libman sac’s endocarditis), serosa
lupus nephritis
minimal mesangial mesangial proliferative focal diffuse membranous advanced sclerosing
lupus nephritis classes
minimal mesnagial - immune complexes
mesnagial proliferative - mesangium hypercellularity and matrix increase
focal - mesangial swelling and proliferation, neutrophils and fibrinoid deposits (in capillaries) hematuria, proteinuria/ red cell casts/ renal insufficiency
diffuse - half or more glomeruli affected, subendothelial immune complexes, thickened wire loop capillary walls/ glomerulosclerosis/ hematuria, proteinuria/ hypertension/ renal insufficiency
membranous - subendothelial immune complexes in capillaries, proteinuria, NEPHROTIC syndrome
advanced - 90% glomeruli - end stage
SLE symptoms
skin - malar maculopapular butterfly rash
photosensitivity
dermoepidermo immune complexes
joints - swelling, mononuclear cells
cns - atherosclerosis/ thrombosis - cerebral angiopathy - infarcts - neuropsychiatric disorders
spleen - onion skin lesions (penicillary artery fibrosis)
pericardial and pleural serositis
libman sacks endocarditis - mononuclear cells infiltrate, - verrucous endocarditis (warty deposits on leaflets)
SLE treatment
anti CD20 antibody - rituximab
blocking growth factors
CREST
limited scleroderma
calcinosis, Raynaud’s phenomenon, esophgeal dysmotility, sclerodactyly, telangiectasia
systemic sclerosis
limted
diffuse
Sjorgen syndrome
lacrimal and salivary gland effected -
keratoconjunctivitis sicxa and xerostomia
systemic sclerosis - skin
epidermis: skin capillaries thicken then fibrosed - occlude - skin epidermal atrophy - extremities - progress centrally.
dermis: edema of dermis - dermis fibrosis (epidermis thins and appendages atrophy occurs) - dermal vessels fibrosis
CD 4 T cells
joint motion limitations
drawn mask face
git scleroderma
mostly esophagus affected inflexibility GERD atrophy and fibrosis of muscularis barret's metaplasia
small intestine - loss of villi, microvilli, malabsorption syndrome
musculoskeletal system in scleroderma
synovial hyperplasia and inflammation
inflammatory myositis
Lungs
endothelial activation
pulmonary vessels - vasospasm - hypertension
fibrosis - interstitium
kidneys scleroderma
intimal thinkening (gp, mucopolysaccharides) intralobular arteries - hypertension in 30% people thrombosis, infarction - fibrinoid necrosis
heart scleroderma
patchy myocardial fibrosis
thickened myocardial arterioles corpulmonale
mixed connective tissue disease
arthritis swelling of hand Raynaud's phenomenon esophagus dysmotility, myositis leukopenia fever lymphadenopathy hyeprgammaglobulinemia
IgG4 related
increased IgG4 producing B cells
all organs involved
B cells - organised germinal centers
T cells- diffused
storiform fibrosis
obliterative phlebitis, tissue eosinophilia
diseases associated with IgG4
mikulicz - salivary and lacrimal
reidel’s thyroiditis retroperitoneal fibrosis
autoimmune pancreatitis
inflammatory pseudotumors
