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FA Vasculitides

Vasculitides

QuestionAnswer
hereditary hemorrhagic telangiectasias - AKA? AKA Osler-Weber-Rendu
AKA Osler-Weber-Rendu. Inheritance? Signs? recurrent nosebleeds, skin discolorations, mucosal telangiectasias, GI bleeds
patient: recurrent nosebleeds, skin discolorations, mucosal telangiectasias, GI bleeds. Dz? osler weber rendu, aka hereditary hemorrhagic telangiectasia. AD inheritance.
raynaud's. what size vessel? MOA? small vessel. dec blood flow to skin due to arteriolar vasospasm in response to cold temp or stress.
raynaud's phenom assoc with MCTD or SLE or CREST. small vessel. dec blood flow to skin due to arteriolar vasospasm in response to cold temp or stress.
Wegener's Granulomatosis. Triad? size vessel? focal necrotizing vasculitis of small vessels, necrotizing granulomas in lung and upper airway, necrotizing glomerulonephritis. May see perforation of nasal septum, hematuria, hemoptysis, chronis sinusitis, otitis media, cough, mastoiditis, dyspnea
marker of wegener's? (Ab?) c-ANCA. large nodular densities on cxr. hematuria and RBC casts.
treatment of wegener's? cyclophosphamide and corticosteroids
other ANCA positives? microscopic polyangiitis, primary pauci-immune crescentic glomerulonephritis, Churg-Strauss syndrome
ANCA: p or c? microscopic polyangiitis, primary pauci-immune crescentic glomerulonephritis, Churg-Strauss syndrome -Describe each, size vessel? All affect small vessels. 1) p-ANCA. Like wegener's but lacks granulomas. 2) paucity of Ab's, limited to kidneys 3) p-ANCA. granulomatous vasc with eosinophilia.
Churg-Strauss granulomatous vasculitis with eosinophilia. affects small vessels that often presents with asthma, sinusitis, skin lesions, and peripheral neuropathy (wrist/foot drop). Can also involve heart, GI, kidneys. p-ANCA.
Sturge-Weber Dz small vessels. congenital vasc disorder that affects capillary sized vessels. seizures, early onset glaucoma, port wine stains (aka nevus flammeus) on face, ipsilateral leptomeningiomas (intracerebral AVM)
what is most common childhood vasculitis? Henoch-Schonlein Purpura
Henoch-Schonlein Purpura - size vessel? describe? precipitated by? Ab's? most common childhood vasculitis. small vessels. triad: skin (rash on butt and legs - palpable purpura), joints (arthralgia), GI (intestinal hemorrhage, abdom pain, melena). Follows URI. IgA immune complexes and IgA nephropathy.
kid has URI and then gets lesions on butt and legs. he has stomach aches, melena, hematuria, and joint pain. what does he have. Henoch schonlein purpura. Follows URI. IgA immune complexes and IgA nephropathy.
Henoch schonlein - if you see multiple lesions, are they of the SAME age or of DIFFERENT ages? Which disease has the other aged lesion? SAME age. Polyarteritis nodosa = different ages of lesions.
Buerger's disease - AKA? aka: thrombangitiis obliterans.
small vessel vasculitides (affect ONLY small vessels) telangiectasia, raynaud's, wegener's, microscopic polyangiitis, primary pauci-immune crescentic GNitis, Churg-strauss, sturge-weber, henoch-schonlein
small and medium vessel vasculitides Buerger's (thrombangiitis obliterans), kawasaki, polyarteritis nodosa
medium and large vessel vasculitides takayasu's, temporal (giant cell) arteritis
Buerger's disease. what is it? idiopathic, segmental, thrombosing vasculitis of small and medium peripheral arteries and veins. seen in HEAVY SMOKERS.
who get's buerger's disease? heavy smokers, young men.
symptoms of buergers. worst complication? intermittent claudication, superficial nodular phlebitis, raynaud's, severe pain. may lead to gangrene and autoamputation of digits via painful ischemic disease.
treatment of buerger's smoking cessation
medium vessel diseases cause ___ / ___ of arteries ___ size vessels cause thrombosis / infarction of arteries
kawasaki disease - what is it? acute, self-limiting, necrotizing vasculitis of small and medium vessels in infants/children. asian.
who get's kawasaki's? infants, children, asians.
which diseases have granulomas? wegener's (vs. microscopic polyangiitis does NOT have these). churg-strauss (with eosinophilia), Takayasu's, temporal arteritis
which diseases are thrombosing? buerger's. PAN = fibrinoid necrosis.
which ones are necrosing? wegener's. microscopic polyangiitis? kawasaki. PAN (fibrinoid necrosis).
symptoms of kawasaki's fever, conjunctivitis, changes in lip/oral mucosa ("strawberry tongue"), lymphadenitis, desquamative rash. may develop coronary aneurysms.
which one causes coronary aneurysms? kawasaki. acute, self-limiting, necrotizing vasculitis of small and medium vessels in infants/children. asian.
desquamative rash, conjunctivitis, changes in lips/oral mucosa ("strawberry tongue") kawasaki
treatment of kawasaki IV Ig, aspirin
polyarteritis nodosa. what is it? immune-complex mediated transmural vasculitis with fibrinoid necrosis. small and medium vessels.
immune complex mediated vasculitides? henoch-schonlein, PAN
PAN - associated with ___ ___ - associated with Hep B (seropositive in 30% of patients.)
PAN: Lesions are of ___ (same/different) ages. different ages of lesions
PAN typically involves vessels in what organs? renal and visceral. NOT pulmonary vessels.
PAN does NOT involve ___ vessels. (which organ?) ___ does NOT involve PULMONARY vessels (involves renal and visceral)
symptoms of PAN fever, weight loss, malaise, abdominal pain, melena, headache, myalgia, hypertension, neurologic dysfxn, cutaneous eruptions, multiple aneurysms and constrictions on arteriogram (nodules destroy IEL and media)
Takayasu's arteritis - aka? aka pulseless disease
what is takayasu's? granulomatous thickening of aortic arch and/or proximal great vessels. affects medium and large vessels.
findings in takayasu's high ESR, asian females <40 y/o. FAN MY SKIN On Wednesday: Fever, Arthritis, Night Sweats, MYalgias, SKIN nodules, Ocular disturbances, Weak pulses in upper extremities
epi of takayasu's female <40 y/o. cannot feel or weak pulse in upper extremities.
temporal arteritis - what is it? affects medium and large arteries (esp carotid). focal granulomatous inflammation. usually affects elderly females.
epi of giant cell arteritis elderly females. usually affects carotid branches. most common vasculitis affecting med and large arteries.
___ is the most common vasculitis affecting medium and large arteries giant cell (temporal) arteritis
symptoms of giant cell (temporal) arteritis unilateral headache, jaw claudication, impaired vision (occlude ophthalmic artery that may lead to irreversible blindness). High ESR. Half patients have systemic involvement and polymyalgia rheumatica.
Irreversible blindness giant cell arteritis.
polymyalgia rheumatica giant cell (temporal) arteritis
unilateral headache, jaw claudication giant cell (temporal) arteritis
fever, arthritis, night sweats, nodules, weak pulses in upper extremities takayasu
asian female < 40 y/o takayasu
Hepatitis B associated PAN
PAN vs. Henoch-Schonlein Both: abdom pain, melena, immune-complex mediated, involves renal. PAN: lesions of diff ages, fibrinoid necrosis, htn, neuro dysfxn, myalgia, NOT pulm vessels H-S: kids, follows URI, palpable purpura, arthralgia, IgA nephropathy, intestinal hemorrhage
male, heavy smoker Buerger's (thrombangiitis obliterans)
kid with rash Henoch-schonlein
kid with coronary aneurysm kawasaki
vasculitis with eosinophilia churg-strauss
c-ANCA vs. p-ANCA c: wegener's p: microscopic polyangiitis, Churg-Strauss
gangrene and autoamputation of digits buerger's
kid, skin involvement, joints, GI henoch-schonlein
port-wine stain sturge-weber. also will see ipsilateral leptomeningial angiomatosis (intracerebral AVM), seizures, glaucoma
peripheral neuropathy (foot/wrist drop) + asthma + granuloma churg-strauss
limited to kidney. low antibodies. pauci-immune crescentic glomerulonephritis.
hemoptysis, hematuria, perforated nasal septum wegener's
large nodular densities on cxr, and RBC casts in urine wegener's
lung and (upper/lower) airways in wegener's? lung and UPPER airway in ___
churg-strauss can also involve __, __, __ __-__ can also involve heart, GI, and kidney
Created by: pecanpie792