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FA Vasculitides
Vasculitides
| Question | Answer |
|---|---|
| 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 |
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pecanpie792
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