autoimmune disorders Flashcards
what type of hypersensitivity is associated with SLE ?
Type 3
what are the serological tests used for the identification of SLE ?
ANA non specific
Anti DsDNA specific
Anti smith
what is anti smith directed against ?
snRNP
what are all the penias in SLE due to ?
type 2 hypersensitivity reaction
which type of lupus nephropathy is the most common type in SLE ?
diffuse proliferative GN
which presents as nephritic syndrome
what type of endocarditis is associated with SLE ?
libman sacks endocarditis
what is very distinctive about libman sacks endocarditis ?
affects both sides of the valve
what are the three clinical consequences of antiphospholipid antibody ?
antiphospholipid syndrome
increased PTT
false positive syphillis
what are the three types of antiphospholipid antibodies ?
anti cardiolipin
lupus anticoagulant
anti b2 glycoprotein
what other disease is associated with anti-cardiolipin ?
syphillus
which antibody is associated with a false elevation of PTT ?
lupus anticoagulant
which antibody is associated with drug induced lupus ?
anti histone ab
which drugs are associated with drug induced lupus ?
isoniazid
hydralaziine
procinamide
what is the pathophysiology in rheumatoid arthritis ?
inflammation of the synovium due too TNF and IL 6
Type 3 HS
causing the formation of pannus
what is the classical presentation of RA ?
symmetrical joint inflammation
MCP
PIP
but the DIP is spared
Ulnar deviation
Swan Neck deformity - Hyperextended PIP , hyper flexed DIP
what antibodies are associated with RA ?
RF ( seroo positive)
anti-ccp
whats the target for RF ?
Fc portion of IgG Ab
what HLA is associated with RA ?
HLA-DR4
what are the long term complications of RA ?
osteoporosis
increased risk of coronary heart disease
secondary amyloidosis
what drugs are used for the treatment of RA ?
steroids
NSSAIDS
DMARDS
what is the function of infliximab ?
a monoclonal antibody which is directed against TNF alpha
what is the problem with the se of infliximab ?
there is a risk of reactivation off TB so a PD test must be done before administration
what is thee function of etanercept ?
acts as tumor necrosis factor inhibitor
what is felty syndrome ?
rare complication of RA
splenomegaly
anemia
thrombocytopenia
arthiritis
what is thee pathophysiology of scleroderma ?
fibroblast activation leading to excess collagen deposition
what are the 2 clinical syndromes associated with scleroderma ?
diffuse
limited CREST
Calcinosis
Raynauds
Oesophageal motility
sclerodactyly
telengectasia
what is the main risk associated with limited scleroderma ?
pulmonary disease
how is renal crisis in association with diffuse scleroderma treated ?
ACE inhibitor
what are the antibodies associated with diffuse scleroderma ?
anti scl 70
which is ant topoisomerase 1
anti RNA polymerase II
which antibody is associated with limited scleroderma ?
anti centromere
if a patient with scleroderma starts presenting with fatigue itching and jaundice what is the most likely diagnosis ?
primary biliary cirrhosis
what are the antibodies associated with sjogren syndrome ?
anti ro
anti la
biopsy off salivary gland off patient with sjogren ?
lymphocytic sialadenitis
how do we treat sjogren ?
muscarinic agonists
what is the test for thee sicca symptoms of sjogren ?
schrimer test
salivary gland scintigraphy
if a patient with sjogren presents with persistent unilateral swelling what is this a sign off ?
most probably a b cell lymphoma
what are the large vessel vasculitis ?
temporal arteritis
takayasu arteritis
what are the medium vessel vasculitis ?
polyarteritis nodosa
kawasaki disease
beurgers disease
what are the small vessel vasculitis ?
churg strauss
wegners granulomatosis
microscopic polyangitis
henoch- schonlein purpra
what is the main symptom associated with vasculitis ?
palpable purpra
do not blanch on pressure
what are thee consequences of untreated temporal arteritis ?
blinndness due to affection of the occcular artery
where is the affection in takayasu arteritis ?
aortic arch with granulomatous thickening
what is thee presentation of takayasu arterritiis ?
weak unilateral pulses
BP difference between arm and legs
what is thee presentation in kawasaki disease ?
young asian kids
strawberry tongue
what is the feared complication in kawasaki disease ?
coronary aneurysm
what is the treatment for kawasaki ?
IVIg
aspirin
what disease is commonly confused with kawasaki diseease ?
scarlet disease
but the difference is that thee patent present with a sore throat before thee presentation of the strawberry tongue
what is thee presentation of reye syndrome ?
encephalopathy
confusion
often coma
after the administration of reye syndrome
what group is more liable to get beurgers disease ?
male smokers
thromboangitis obliterans
what disease is associated with polyarteritis nodosa ?
hepatitis B
what is the pathophysiology off polyarteritis nodosa ?
type 3 Hypersenitivty
what sign is oft3en seen with polyarteritis nodosa ?
rosary sign seen on arteriogram
what is the other name for pANCA and ANCA ?
pANCA - PR3
cANCA - MPO
which vasculitis is pANCA and which are cANCA ?
cANCA - Wegner
pANCA - Churg Straus
microsccopic polyangitis
what is thee presentation of churg strauss ?
asthma for thee first time as an adult
bizarre presentation of asthma
sinusitis and neuropathy
what labs are associated with churg strauss ?
high eosinophils
high IgE
p-ANCA
what is the presentation off wegners granulomatosis ?
triad off hemoptysis
sinusitis
otitis media
inflammation of the upper airway and kidney involvement
what is thee classic case of polyarteritis nodosa ?
a patient with hep b that starts presenting with neuropathy and renal failure
what is seen on biopsy of polyarteritis nodosa ?
fibrinoid necrosis
what is the pathophysiology fo henoch schonlein purpra ?
IgA associated nephropathy
with C3 complement deposition
what i9s the classic presentation of henoch scchonlein purpra ?
childhood vasclitis
URI
Melena
what do all ANCA diseases have in common ?
they all have some form of lung and renal involvement
the renal involvement is cresenteric RPGN
what is seen on IF in cresenteric RPGN ?
paucimmune ( lack of Ig )
what is the presentation off microscopic polyangitis ?
just like wegner but no upper airway affection
what is the pathophysiology of goodpasteur syndrome ?
type 2 hypersenitivity
anntibodyy too collagen type 4
anti basement membrane
immunoflurosence is positive