Immunopathology II Flashcards
how does acute GVHD affect skin
rash exfoliation
how does acute GVHD affect GI
ulcerative gastroenteritis
how does acute GVHD affect liver
bile duct necrosis
what is the mechanism of acute GVHD
donor cytotoxin T cells or cytokines from helper T cells destroy epithelial celsl
when does chronic GVHD happen
after resolution of acute GVHD or without acute phase
what does the morphology of chornic GVHD miminc
systemic sclerosis
what does chronic GVHD cause
generalized fibrosis of GI, Skin, LIver, and broncholes of lunch
what critieria is required for an autoimmune diseaes
immunological reaction to self=Ag or native tissue
reaction is primary to pathogensis
no other etiology
polymorpims is what gene is often implicated in autoimmunity
PTPN-22
how do microbes influence auto-immunity
increase expression of APC co-stimulatory molcules, non-soecific B and T cell stimulation
what is the prototypical multisystem disorder
SPE
What is the ACR diagnositic creteria for SPE
malar rash discoid rash photsensitivty oral ulcers arthritis serositis renal diorder neurologic hemotologic antinuclear antibodies
mala rash
butterfly rash
discoid rash
scaely
serositis
ST elevation of EKG
what is the hematologic disorder seen in SPE pts
hemolytic anemia or leukopenia or lyophopeia or thrombocytopenia
what locus is associated with SPE
HLA-DQ
what are the most common clinical signs in SPE pts
hematologic arthritis skin fever fatique weight loss renal
what is the hallmark of lupus
antinuclear antibodies
how do you test for ANA
fluorescnese
homogenous ANA pattern indicates
RA or drug induced SLE
rim pattern of staining indictaes
dsDNA, SLE active flares
speckled ANA pattern inidcates
anti-smith Ab of SLE, systemic sclerosis, sjogren’s syndrome
nucleolar ANA pattern inidcates what
SLE, systemic sclerosis, CREST
centromere pattern inidcates
systemic sclerosis, CREST
type II mechanism in lupus
against blood cells, causes frequent hematologic abnormalites
type III mechanism in lupus
visceral lesions
how are joints affects in lupus
synovitis without deformity
how are joints affects in lupus
synovitis without deformity
acute lupus vascular changes
vasculitis with fibrinoid necrosis of arteries/arterioles in any tissue
chronic lupus vascular changes
layered fibrous thickening - onion skin looking
lupus nephritis
prototype of immune complex glomerulnephritis
what are the 5 patterns of lupus nephritis
minimal changes
mesangial CN with increase in cells and matrix
membranous
diffuse GN with proliferation of endothelial, epithelial, mesagnial celsl
membranous glomerulonephritis
thickening of capillary loops
acute form of serositis
exudation of fibrin
chronic form of serositis
proliferation of fibrous tissue, thickening membranes, adhesions
heart problems often seen in lupus
frequent pericarditis
occasional mycoarditis
nonbacterial endocaridits
vegetative endocarditis in rheumatic heart disease
small, warty vegetations along lines of closure of balbe leaflets
infection endocarditis vegative endocarditis
large, irregular destructive masses of valve cusps, can extend to chordae
vegetative endocarditis in nonbaterical thrombotic endocarditis
small, bland vegetations at the line of closure
vegetative endocarditis in libman=sacks endocarditis
small-medium sized vegetrations on either/both sides of valve leaflets
acute lung problems with lupus
penumotisis with alveolar damge, edema, and hemorrhage
chronic lung problems with lupus
interstitial and vascular fiborsis causing pulomary fiborsis and pulmonary hypertension (restrictive lung disesase)
common lupus CNS problems
seizures, focal neurologic deficit, neuropsych
what tends to be the COD with lupus
renal faiure, infection, CAD
chronic discoid LE
variety of skin lesions without systemic features, SLE can develop but rare
subacute cutaneous LE
diffuse superifical, nonscarring photosenstive lesions
mild systemic disease
in between form of lupus
drug induced lupus
mutiple organ involvment, no renal or SNS pathology
what drugs can cause lupus
d-penicillamine, procaiaminde, hydralazine, isonizid
sjogren’s syndrome
autoimmune destruction of exocrine glands - mostly lacrimal and salivary
pathogenesis of sjogrens
CD4+T cells against glandular epithelial self-Ag initate the disorder, can be induced by viral infection
sjogens’ morphology
follicle formation with germinal centers
ductal epithelial hyperplasia
acinar atrophy, fat replacement of parenchyma
systemic sclerosis
autoimmune disorder characterized by chronic inflammation, destruction of small vessels and progressive tissue firosis
invovledment of what in systemic sclerosis causes the most morbidy and mortiality
GI, kidney, heart, lung, muscoskeletal
what does CREST stand for
calcinosis raynaud's phenomenon esophageal dysfunction sclerdactylyl telangiectasis
raynaud’s phenomen
spasm of blood vessels in response to cold or stress
sclerodactylyn
thickening and tightening of skin on fingers and hands
telangiectaias
dilation of capillaries causing red makes on surface of skin
diffuse varient of systemic sclerosis
widespread skin involemnt at onset
early vesiceral invovlemnet
rapid progression
Ab to DNA topiosermiase I
early skin changes in diffuse varient of systemic sclerosis
edmea, lyphocyte infiltrates
late skin changes in diffuse varient of systemic sclerosis
claw fingers, mask faces
esophagus changes in systemic sclerosis
collagenization and fibrosis of muscularis, can get reflus/barnett’s
small bowel cances in systemic sclerosis
mucosal thinning, loss of villi/microvilli, malabsorption
early stage of musculoskeletal changes in systemic sclerosis
nondestructive hyperplasia and inflammation of synovium
late stage of muscular skeletal changes in systemic sclerosis
fibrosis of synovial and periarticular CT
renal changes in systemic sclerosis
thickening of interlobular arteries by concentric proliferation of intimal cells, deposition of collagenous or mucinous material and hyaline changes
pulomary changes in systemic sclerosis
alveolar fiborsis
pulomary hypertension
COD in systemic sclerosis
renal, cardiac, pulmonary, GI dysfunction or failure
rheumatoid arthritis
systemic autoimmune inflammatory disorder
synovitis in rheumatoid arthrits
nonsuppurative, prolifeative, destructive
pannus formation
granulation tissue, synovial and inflammatory cell, fibrous CT
how does RA affect hands
diffuse osteopenia
marked loss of joint spaces
ulnar drift of fingers
osteopenia
loss of joint space
COD of RA
disease complications of amyloidosis, vasculalitis
complications of drug therapy
juvenile idiopathic arthritis often affects
large joints
systemic features of juvenile idiopathic arthristis
pericarditis
mycoarditis
pulmonary fibrosis
glomerulonephritis
still’s disease varient
febrile illnesss with hepatosplenomegaly, rash, incraesed WBC count
mixed connective tissue disease
clinical syndrome with overlapping features of SLE and Systemic sclerosis
what ab is affect in MCTD
anti-U1RNP - to ribonucleoproten
what do you treat MCTD with
steroids