First Aid Vasculitis Flashcards
large vessel vasculitis
Temporal (giant cell) arteritis
Takayasu arteritis
medium vessel vasculitis
Polyarteritis nodosa (PAN) Kawasaki disease Buerger disease (thromboangiitis obliterans)
small vessel vasculitis
Granulomatosis with polyangiitis (Wegener)
Microscopic polyangiitis
Churg-Strauss
Henoch-Schonlein purpura
Temporal (giant cell) arteritis presentation
unilateral headache (temporal artery), jaw claudication, may lead to irreversible blindness due to ophthalmic artery occlusion, associated with polymyalgia rheumatica
Pathology/lab findings with giant cell arteritis
most commonly affects branches of carotid artery
focal granulomatous inflammation
increased ESR
how do you treat giant cell arteritis?
high dose corticosteroids prior to temporal artery biopsy to prevent vision loss
Presentation of Takayasu arteritis
Asian females less than 40 years old
weak upper extremity pulses, fever, night sweats, arthritis, myalgias, skin nodules, ocular disturbances
Pathology/lab findings with Takayasu arteritis
granulomatous thickening and narrowing of aortic arch and proximal great vessels, increased ESR
how do you treat takayasu?
corticosteroids
presentation of PAN?
hepatitis B seropositivity in 30% of patients
fever, weight loss, malaise, headache, abdominal pain, melena, hypertension, neurologic dysfunction, cutaneous eruptions, renal damage
Pathology/labs with PAN?
typically involves renal and visceral vessels not pulmonary arteries
immune complex mediated
transmural inflammation of the arterial wall with fibrinoid necrosis
innumerable microaneurysms and spasm on arteriogram
treatment of PAN
corticosteroids, cyclophosphamide
Kawasaki disease presentation
Asian children less than 4 years old
fever, cervical lymphadenitis, conjuctival injection, change sin lips/oral mucosa (strawberry tongue), hand-food erythema, desquamating rash
Pathology/labs with kawasaki disease
may develop coronary artery aneurysms -> MI, rupture
treatment of kawasaki disease
IV immunoglobulins and aspirin
Buerger disease presentation
heavy smokers, males less than 40 years old
intermittent claudication may lead to gangrene, autoamputation of digits, superficial nodular phlebitis
raynaud phenomenon is often present
treatment of buerger disease
smoking cessation
presentation of granulomatosis with polyangiitis (Wegener)
URT: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis
LRT: hemoptysis, cough, dyspnea
renal: hematuria, red cell casts
pathology/labs with granulomatosis with polyangiitis
triad: focal necrotizing vasculitis, necrotizing granulomas in the lung and upper airway, necrotizing glomerulonephritis
CXR: large nodular densities
antibodies with granulomatosis with polyangiitis
PR3-ANCA, c-ANCA
treatment of granulomatosis with polyangiitis
cyclophosphamide, corticosteroids
presentation of microscopic polyangiitis
necrotizing vasculitis commonly involving lung, kidneys, and skin with pauci-immune glomerulonephritis and palpable purpura (similar to wegeners but w/o nasopharyngela involement)
antibodies with microscopic polyangiitis
MPO-ANCA/p-ANCA
how do you treat microscopic polyangiitis
cyclophosphamide and corticosteroids
Churg-Strauss presentation
asthma, sinusitis, palpable purpura, peripheral neuropathy
can also involve heart, GI kidneys (pauci-immune glomerulonephritis)
Churg-Strauss pathology/labs
granulomatous, necrotizing vasculitis with eosinophilia
MPO-ANCA/p-ANCA, increased IgE levels
Henoch-Schonlein purpura presentation
most common childhood vasculitis follows URI
skin: palpable purpura on buttocks/legs
arthralgias
GI: abdominal pain, melena, multiple lesions of same age
Henoch-SChonlein purpura pathology/labs
vasculitis secondary to IgA complex deposition
associated with IgA nephropathy
