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)
Hep B management PAn
plasmapheresis to increase chance of seroconversion
antivirlas
cause of death in PAN
uncontrolled vasculitis, infection complication due to tx induced immunosuppression, vascular complication
worse prognosis if pt has severe underlying dz
systemic dz characterized by skin induration and thickening
scleroderma
+ chronic inflammatory infiltration in numerous visceral organs and vasculature
scleroderma epidemiology
AFRICAN AMERICAN women
30-50
pathogenesis of scleroderma
- overproduction of collagen and connective tissue proteins = fibrosis
- vasculitis of small vessels with obliteration
- abnormal activation of humoral and cellular immune systems
scleroderma skin changes
thick and indurated first distally then spreads proximally
sharp beak like profile w/few wrinkles
sparse hair, digital ulceration, loss of joint creases, limited ROM
thinning of lips, shortened fingers (bone reabsorption)
pathophysiology of scleroderma
endothelial cells transform to myofibroblasts = fibrous tissue within vasculature (decreased ability to constrict and dilate)
production of cytokines by myofibroblasts = fibrosis, number of autoAbs produced
categories of skin changes
limited cutaneous (limited to distal extremities)
diffuse cutaneous (skin thickening of turn, upper arms, thighs)
non cutaneous symptoms of scleroderma
GI vascular pulmonary renal ENT CREST
GI scleroderma
loss of LES = Gerd and aspiration
atrophy of peristaltic muscles = constipation, diarrhea, bacterial overgrowth and malnutrition
vascular scleroderma
raynauds phenomenon (ALMOST ALL)
finger tip ulceration
pulmonary scleroderma
pulmonary HTN
restrictive lung dz
RENAL scleroderma
HTN
Renal iris
chronic renal insufficiency
cardio scleroderma
CHF (myocardial fibrosis)
conduction system dz
syncope (arrhythmia)
ENT scleroderma
SICCA
poor dentition, loss of teeth
hoarseness
CREST syndrome
type of scleroderma
Calcinosis cutis (Ca deposits) Raynaud phenomenon Esophageal dysmotility Sclerodactyly Telangioectasias
renal crisis scleroderma
accelerated HTN, oliguria, HA, dyspnea, edema, rapidly rising serum Cr
diffuse, rapid skin involvement have highest risk (20-25%), BLACKS, MEN
preventing renal crisis scleroderma
not treated = kidney failure
monitor BP and serum Cr
AVOID corticosteroids
natural history scleroderma
typical pt begins by noting Raynaud’s phenomenon
over months to years, skin thickening of digits, puffiness/edema, unresponsive to diuretics and morning stiffness develops
work up scleroderma
- ANA +
- topoisomerase I abs (absent in limited, + in diffuse)
- anti-centromere abs (absent in diffuse)
- PFTs and HRCT of chest
- Transthoracic echo/R Heart Cath for pulmonary HTN
- Esophageal studies
tx of scleroderma
symptomatic management
Non-DHP CCB for raynauds
Meds for pulmonary HTN
pregnancy scleroderma
high risk, esp if <4 yrs of diagnosis
mortality scleroderma
12 yrs following diagnosis
poorer prognosis: young, rapid progression, lung/gi/cardiac involvement
pulmonary HTN prognostic indicator
