vasculidities Flashcards
what are the key features of microscopic polyangitis
- affects kidneys
- causes pauci-immune crescentic glomerulonephritis
- p-ANCA+
what is p-ANCA? What is the antigen and what is the antibody?
perinuclear anti-neurophil cytoplasmic antibodies
antigen is myeloperoxidase
antibody is MPO-ANCA
what is c-ANCA? What is the antigen and what is the antibody?
cytoplasmic anti-nutrophil cytoplasmic antibodies
antigen is proteinase 3 (PR3)
antibody is PR3-ANCA
what are the key features of granulomatosis with polyangitis
focal necrotizing vasculitis with granulomas in the lung and upper airway
glomerulonephritis
c-ANCA
NOTE:differentiate from goodpastures by involvement of upper airway in wegners
what is the treatment for granulomatosis with polyangitis?
cyclophosphamide and corticosteroids
what are the key features of churg-strauss syndome?
granulomatosis with eosinophilia (multinucleated giant cells with eosinophils) and ASTHMA/sinusitis/skin lesions/peripheral neuropathy (foot drop/wrist drop)
+pANCA
what are the key features of henoch-schonlein purpura?
most-common form of systemic vasculitis in childhood
- rash on buttocks and legs made up of PALPABLE PUPURA
- arthralgias (knees)
- abdominal pain with melana(intestinal hemorrhage)
- renal disease (deposition of IgA immune complexes - IgA nephropathy) with nephritic hematuria
OFTEN OCCURS FOLLOWING UPPER RESPIRATORY INFECTION (IgA nephropathy)!!
what are the key features of polyarteritis nodosa?
medium vessel vasculitis that involves kidneys and visceral organs but spares lungs - can have abdominal pain melana, myalgia
associiated with hepB/hepC
NEGATIVE FOR ANCA
what is mucocutaneous lymph node syndrome?
kawasaki’s disease
necrotizing vasculitis seen in asiean infants
fever that lasts >5 days + CRASH symptoms
conjunctuvitis rash on trunk adenopathy strawberry tounge hands and feet - erythematous desquamation
what is a major concern for kawasaki diase?
coronary aneurism
can lead to thrombosis and MI
be prepared to make ID from coronary angiogram
what is treatment for kawasaki disease?
IV immunoglobulin (goal is to flood system with immunoglobulin and cause feedback-suppression of immune responses to decrease production of auto-antibodies) and asprin
What is another name for Burger disease?
thromboangiitis obliterans
vasculitis of medium arteries seen in male smokers in their 40’s
intermittent claudication
superficial nodular phlebitis
can lead to gangreen in fingers
what is another name for giant cell arteritis?
temporal arteritis
affects branches of EXTERNAL carotid arteries (e.g. temporal arteries)
who is predominantly affected by temporal arteritis? what is the classic presentation?
classicly presents in elderly women as unilateral headache with jaw-claudication
occlusion of opthalmic artery can lead to vision loss or irriversible blindness
may have palpable temporal artery
may also have polymyalgia rhumatica (muscle and joint pain)
dx is with temporal artery biopsy
pt presents with unilateral headache and jaw pain with chewing and elevated ESR. What is Dx?
temporal arteritis
what’s another name for takiyatsi disease?
pulseless disease
due to granulomatous inflamation of arteries of the aortic arch
what are the key features of takiyatsi arteritis?
young asian women in teen
poor pulses in extemities
granulomatous inflamation of arteries of the aortic arch
elevated sed rate
vasculitis is 2 year old asian female
kawasaki
vasculitis in 20 year old asean female
takiyatsu
vasculitis associated with asthma
churg strause
most likely vasculitis in young male smoker
burger (thromboarteritis obliterans)
vasculitis associated with polymylagia rheumatica
temporal arteritis
vasculitis associated with IgA nephropatrhy
henoch-schloch purpura
vasculitis associated with Hep B
polyarteritis nodosa
vasculitis associated with jaw claudication and vision loss
temporal/giant cell arteritis
vasculitis associated with strawberry tongue
kawasaki’s disease
vasculitis associated with desquamation of hands/feet
kawasaki’s disease
vasculitis associated with poor pulses in the arms
takiyasu arteritis
vasculitis associated with palpable purpura on the legs
henoch-schlochen purpura
vasculitis associated with kidney and GI involvement but spares the lungs
polyarteruitis nodosa
HEPATITIS!!!
vasculitis of the kidney, upper airway and lungs
granulomatosis with polyangitis
what’s the treatment for renauds?
dihydropyridine calcium channel blockers (e.g. nefidipine)
what is sturge weber disease?
congential disorder that affects capillaries causing AV malformation
princply presents as leptomeningeal angioma
purplish port-wine stain along opthalmic division of trigeminal nerve
associated with seizures, glocoma, tardism
what is a strawberry hemangioma?
benign hemanioma of cutaneous suface of capillaries seen in infants - spontaneous regresses in childhood (gone by age 8)
what is a cherry hemanioma?
benign hemanioma of cutaneous surface capillaries - does not regress very common on most people
what is a pyogenic granulmoma>
raised capillary hemanioma associated with trauam and pregnancy
looks like red-polyp on skkin - frequently ulcerates and bleeds and tend to be on lips an gums
what is a glomus tumor?
benign painful tumor under the fingernails
what is bacillary angiomatosis? what is it associated with?
benign but can be mistaken for karposi sarcoma because looks like multiple raised puplish spots
caused by bartonella henselae - cat scratch disease in immunocompromised
what vascular tumor is associated with prior radiation therapy?
angiosarcoma - often face, scalp, neck or breast
what is a potential consequence of perisisten lymphadema?
lymphangiosarcoma
vasculitis associated with weak pulses in upper extrmeities
takiyatsu
vasculitis associated with necrotizing granulomas of lung and necrotizing glomerulonephritis
granulomatosis with polyangitis
vasculitis associated with necrotizing immune complex inflammation of viseral/renal vessels
polyarteritis nodosa (spares lungs, -ANCA)
vasculitis associated with young male smokers
buerger ddisease
vasculitis associated with young asian women
takayasu disease
vasculitis associated with young asthmatics
churg-strauss
vasculitis associated with infants and young children; involves coronary arteries
kawasaki
most common vasculitis
temporal arterisit
vasculitis associated with hep B
polyarteritis nodosa
vasculitis associated with occlusion of opthalamic artery that can lead to blindness
temporal arteritis
vasculitis associated with perforation of the nasal septum
wegners
vasculitis associated with unilateral headache with jaw claudication
temporal arteritis
benign, raised, red lesion about the size of a mole in older patients
cherry hemangioma
raised, red area present at birth increases in size initially then regresses over months to years
strawberry angioma
lesion caused by lymphoangiogenic growth factors in HIV patient
karposi-sarcoma
polypoid red lesion found in prgnancy or after trauma
pyogenic angioma
benign, painful red-blue tumor under fingernailos
glomus tumor
cavernous lymphangioma associated with turner syndromwe
cystic hygroma
skin papule in AIDS patients caused by bartonella
bacilliary angiomatosis
what is the treatment for bueger disease?
smoking cessation
what is the trwatment for temporal arteritis
high dose steroids
