Rheum D/O 3 Flashcards
PAN
systemic vasculitis
necrotizing inflammatory lesions, medium sized and small muscular arteries ESP at bifurcations
vasculitis of PAN results in
micro aneurysm formation, aneurysmal rupture with hemorrhage, thrombosis, organ ischemia or infarction
what arteries does PAN like?
small and medium sized muscular arteries (esp. at bifurcation)
PAN patho
inflammation in intima and progresses to include entire arterial wall
aneurysm development in weak vessel = hemorrhage and rupture risk + thrombi development
proliferation of intimal or media result in obstruction and subsequent tissue ischemia/infarction
PAN spares…
LARGE vessels, SMALLEST vessels, venous system, pulmonary vasculature
PAN MC affects
skin, joints, peripheral nerves, gut and kidney
associated diseases
HEP B***
HCV, VZV, hIV, parvo, CMV, bacterial infection
rheum dz
PAN and Hep B
occur at any time during infection
MC
onset of PAN
subacute
affects multiple organ systems
PAN systems presentation (8)
- opthalamic
- cardiac
- Renal - arteritis w/o glomerulonephritis (no Casts)
- peripheral neruopathies
- CNS (TIA, CVA)
- skin - livedo reticular
- GI
- constitutional (non specific)
LUNGS SPARED
characteristic rash PAN
tender erythematous nodules with central ulcercation
PAN work up
ESR/CRP elevated, negative AutoAbs
study of choice: angiography
confirm: tissue bx of cutaneous lesions, muscle, vessels
tx of PAN
- steroids
- cyclophosphamide
- management of underlying dz
steroids and PAN
usually start high and taper early (last at least a year)
cyclophosphamide and PAN
severe or steroid refractory dz
significant toxicity
DO NOT USE w/Hep.B pts (increases viral replication)