Vasculitides
Terms
undefined, object
copy deck
- Takaysu
-
pulseless; asian women
thickening of proximal aorta
SX (FAN MY SKIN on Wednesday)
Fever, arthritis, night sweats, myalgia, skin nodule, ocular disturbances, weak pulse
**Med and Large arteries - Temporal arteritis
-
elderly (esp fem); unilateral headache
tx: steroids
Sx may cause proximal muscle pain (polymyalgia rheumatica)
*Sig increased ESR
**Large/med arteries - Kawasaki
-
acute, self-limiting dz of kids/babies
-necrotizing vasculitis
-fever, congested conjunctiva, changes in lips/oral mucosa, coronary artery aneurysm
**mostly small arteries (also med, large) - Polyarteritis Nodosa (PAN)
-
pANCA, fever, wt loss, abd pain, HTN, some ass'd w/ Hep B
-necrotizing degeneration of media-> aneurysms
-cotton-wool spots, palable purpura
TX: steroids, asathioprine, +/- cyclophosphamide
inc. ESR
**Sm/med arteries -
Buerger's
AKA Thromboarthritis obliterans -
Jewish men who smoke
-cold, pale limb, Raynaud's, may -> gangrene
**Sm/med arteries - Churg-Strauss
-
like PAN, but w/ eosinophilia and asthma
(Sm/Med arteries) - Wegner's
-
C-ANCA; necrotizing granulomatous lesions in lung and kidney
Sx: cough, sinus ulcers and perforation of nasal septum, RBC casts in urine
-CXR may show nodular densities
TX: cyclophosphamide, steroids, +/-methotrexate
**Sm arteries and veins - Henoch-Scholein Purpura
-
IgA complex mediated inflmn
rash and renal dz in atopic kid ass'd w/ upper resp tract infxn
**arterioles, capillaries and venules - Rendu-Osler-Weber
-
Mormons (AD)
hereditary hemorrhagic telangiectasia
-> epistaxis, GI bleeds
**Venules and capillaries - Kaposi's Sarcoma
-
malignant vascular tumor
HHV8
**visceral vasculature - Von-Hippel-Lindau
-
Hemangiomas of cerebellum, brainstem, retina - also hepatic renal pancreatic cysts
-AD; chrm 3; *Inc Risk Renal Cell Carc
**visceral vasculature