WT - vasculitides Flashcards
large-vessel vasculitis (2)
- giant cell (temporal) arteritis
- takayasu arteritis
medium-vessel vasculitis (3)
- Buerger
- Kawasaki
- Polyarteritis nodosa
small-vessel vasculitis (7)
- Behcet syndrome
- Cutaneous small-vessel vasculitis
- Eos granulomatosis w/ polyangitis
- Granulomatosis w/ polyangiitis
- IgA vasculitis
- Microscopic polyangiitis
- mixed cyroglobulinemia
giant cell arteritis
- elderly females
- @ br. of carotid a.
- HA, jaw claudication, painful chewing
- assoc w/ polymyalgia rheumatica
- inc ESR
histo: granulomatous inflammation of media w/ fragmentation of internal elastic lamina
tx: steroids ASAP to avoid BLINDNESS via ophthalmic a. occlusion
takayasu arteritis
- young, asian woman (<40 y/o)
- granulomatous thickening @ aortic arch/branches
- PULSELESSNESS disease
- BP Dif between arms/legs
- bruits over a.
tx: corticosteroids
Kawasaki disease
(mucocutaneous lymph node syndrome)
- asian child <5 y.o
- @ coronary a.
sx -skin/lips/STRAWBERRY TONGUE -diffuse rash, palms/soles -coronary a. aneurysm -conjunctival injection -cervical adenopathy -fever (presents like an INFECTION)
tx: IVIG, aspirin**
thromboangiitis obliterans
(Buerger’s disease)
- male, smokers
- hand/feet poor BF via vasospasm –> gangrene, autoamputation, superficial nodular phlebitis
- Raynaud’s phenomenon
- segmental thrombosing vasculitis with VEIN and NERVE involve
tx: smoking cessation
polyartitis nodosa
- middle-aged men
- immune complex mediated @ medium vessels (HS II)
- transmural inflammation of med vessel wall (Dif stages)
- most a. EXCEPT lungs
- assoc w/ hep B+, but usu idiopathic
sx
- nerves:motor/sensory deficits
- GI: abd pain/melena
- skin: nodules/purpura (palpable)
- kidneys: renal failure
arteriogram: aneurysms/constrictions (renal - STRING OF PEARLS)
tx: corticosteroids, cyclophosphamide
IgA vasculitis
(Henoch-Schonlein purpura)
- most comm CHILD systemic vasculitis
- follows URI
sx (triad)
- palpable purpura
- arthralgias
- abd pain/intussusception
tx: supportive, CST
Granulomatosis w/ polyangitis
(WeCner’s granulomatosis)
C=NOSE/LUNG/KIDNEY
- @ upper/lower airway
- upper: septum perforation/chronic sinusitis/otitis media/mastoiditis, SADDLE NOSE
- lower: hemoptysis, cough, dyspnea
- renal: hematuria, red cell casts
sx (triad)
- focal necrotizing vasculitis
- necrotizing granulomas in lung/upper airway
- necrotizing glomerulonephritis
dx: c-ANCA (correlates w/ severity)
tx: steroids/cyclophosphamids (relapses COMMON)
microscopic polyangitis
sim to weCners but NO upper airway disease/granulomas
- seen w/ abx use
- necrotizing vasculitis of lung, kidneys, skin w/ PAUCI-IMMUNE glomerulonephritis and palpable purpura
dx: MPO-ANCA/p-ANCA
Eosinophilic granulomatosis w/ polyangitis
(Churg-Strauss)
-granulomatous, necrotizing vasculitis with EOS
- asthma/sinusitis/neuropathy
- eos
- heart/GI/kidneys (PAUCI-IMMUNE glomerulonephritis)
dx: MPO-ANCA, p-ANCA, inc IgE
mixed cyroglobulinemia
- chronic hep C
- presence of cryoglobulins that precipitate when cooled in vitro
- IgG and IgM mixed
sx (triad)
- purpura
- weakness
- arthralgia
+ peripheral neuropathy/glomerulonephritis
dx: IgG, IgM complex deposition
behcet syndrome
- turkish/eastern meditteranean
- HLA-B51
sx
- aphthous ulcers
- genital ulcerations
- uveitis
- erythema nodosum
-flares last 1-4 wk, can be precipitated via HSV or Parvo
cutaneous small cell vasculitis
- immune complex-mediated leukocytoclastic vasculitis
- 7-10d post meds (penicillin, cephalosporins, phenytoin, allopurinol)
- or post infec (HCV, HIV)
sx
- late involvement=systemic vasculitis
- palpable purpura
- NO visceral involve
histo: perivasc inflame w/ fibrinoid necrosis, predominantly neutrophils