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