Vasculitis Flashcards
Basic treatment for all vasculitities
steroids
Types of large vessel vasculitides
temporal (giant cell)
Takayasu
Types of medium vessel vasculitis
Polyarteritis nodosa
Kawasaki
Burger
Small vessel vasculitides
Wegner granulomatosis
microscopic polyangiitis
Chruge-Strauss
Henoch-Schönlein Purpura
Temporal (giant cell) arteritis
biopsy findings
granulomatous: inflammed vessel wall, giant cells, intimal fibrosis
segmental lesions (need long biopsy)
negative Bx does not exclude disease
Presentation of temporal arteritis
headache, visual disturbances (opthalmic artery involvement), jaw claudication
Tx of temporal arteritis
steroids
(Tx if suspected at all! not treating can lead to blindness)
Takaysu arteritis
which arteries does it normally affect?
aortic arch and branches
classically a young Asian female
Physical exam findings of Takayasu arteritis
neurologic symptoms
weak or absent upper extremity pulses
(subclavian branches are blocked off)
Polyarteritis Nodosa
type of vasculitis
medium vessel
necrotizing vasculitis
Organ involvement of polyarteritis nodosa
anything but the lungs
Presentation of polyarteritis nodosa
HTN (renal artery involvement)
abdominal pain with melena–dark stool (mesentaric artery involvement)
neurologic distrubances
skin lesions
What can you find in the serum when you have polyarteritis nodosa?
hepatitis B surface antigen
(HBsAg)
Bx results of polyarteritis nodosa
“string of pearls”
fibrinoid necrosis (weaking of wall causes aneurysms)
Tx of polyarteritis nodosa
cyclophosphamide
corticosteroids
Kawasaki disease
presentation
Asian children < 4 y/o
non-specific Sx: rash on palms and soles, fever, conjuctivitis, enlarged cervical lymph nodes
Biggest complication of Kawasaki disease
coronary artery involvement
thrombosis or aneurysm with rupture
Tx of Kawasaki disease
aspirin
IVIG
Buerger Disease
associated with heavy smoking
autoamputation of digits
Wegner’s granulomatosis
associated serum marker
c-ANCA
cytoplasmic-anti neutrophil cytoplasmic antibody
Microscopic polyangiitis
associated serium marker
p-ANCA
perinuclear-anti-neutrophil cytoplasmic antibody
Churg-Strauss Syndrome
associated serum marker
p-ANCA
Wegner granulomatosis
affected organs
nasopharynx (chronic sinusitis, ulcerations)
lungs (classic: bilateral nodules)
kidneys (renal failure)
Wegner granulomatosis
Bx results
necrotizing granulomas w/ adjacent necrotizing vasculitis
Tx of Wegner’s
corticosteroids
cyclophosphamide
Microscopic Polyangiitis
affected organs
lungs and kidneys
Microscopic Polyangiitis
Tx
corticosteroids
cyclophosphamide
Differences between Wegner and microscopic polyangiitis
Wegers: nasopharynx, lungs, kidneys
microscopic polyangiitis: lungs, kidneys
Wegners: cANCA
microscopic polyangiitis: pANCA
Chrug-Strauss
affected organs
lungs and heart
Churg-Strauss
associated symptoms
eosinophilia
asthma
Chrug-Strauss
Bx
necrotizing granulomatous inflammation w/ eosinophils
Henoch-Schönlein Purpura
defining pathology
vasculitis due to IgA immune complex deposition
type III hypersensitivity
Skin lesions in Henoch-Schönlein
Henoch-Schönlein purpura!
palpable purpura
raised lesions, usually found on buttocks and legs
What organs does Henoch-Schönlein Purpura normally affect
skin (palpable purpura)
kidneys (IgA nephropathy)
GI
Tx of Henoch-Schönlein Purpura
resolves on own usually
steroids if refractory or recurrent
Most common vasculitis in children
Henoch-Schönlein Purpura
Which vasculitis is associated with
p-ANCA
microscopic polyangiitis
Churg-Straus
How to differentiate between
microscopic polyangiitis
and
Churg-Strauss
both have p-ANCA
microscopic polyangiitis affects lungs and kidneys
Churg-Strauss affects heart and lungs
Churg-Strauss is has necrotizine granulomatous lesions
How to differentiate between
Wegner granulomatosis
vs
microscopic polyangiitis
Weger: cANCA
Microscopic polyangiitis: pANCA
Wegner: granulomatous lesions of nasopharynx, lung, kidney
Microscopic polyangiitis: nongranulomatous lesions of lung and kidney
