Rheum D/O 3 Flashcards

1
Q

PAN

A

systemic vasculitis

necrotizing inflammatory lesions, medium sized and small muscular arteries ESP at bifurcations

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2
Q

vasculitis of PAN results in

A

micro aneurysm formation, aneurysmal rupture with hemorrhage, thrombosis, organ ischemia or infarction

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3
Q

what arteries does PAN like?

A

small and medium sized muscular arteries (esp. at bifurcation)

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4
Q

PAN patho

A

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

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5
Q

PAN spares…

A

LARGE vessels, SMALLEST vessels, venous system, pulmonary vasculature

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6
Q

PAN MC affects

A

skin, joints, peripheral nerves, gut and kidney

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7
Q

associated diseases

A

HEP B***

HCV, VZV, hIV, parvo, CMV, bacterial infection

rheum dz

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8
Q

PAN and Hep B

A

occur at any time during infection

MC

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9
Q

onset of PAN

A

subacute

affects multiple organ systems

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10
Q

PAN systems presentation (8)

A
  1. opthalamic
  2. cardiac
  3. Renal - arteritis w/o glomerulonephritis (no Casts)
  4. peripheral neruopathies
  5. CNS (TIA, CVA)
  6. skin - livedo reticular
  7. GI
  8. constitutional (non specific)

LUNGS SPARED

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11
Q

characteristic rash PAN

A

tender erythematous nodules with central ulcercation

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12
Q

PAN work up

A

ESR/CRP elevated, negative AutoAbs

study of choice: angiography

confirm: tissue bx of cutaneous lesions, muscle, vessels

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13
Q

tx of PAN

A
  1. steroids
  2. cyclophosphamide
  3. management of underlying dz
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14
Q

steroids and PAN

A

usually start high and taper early (last at least a year)

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15
Q

cyclophosphamide and PAN

A

severe or steroid refractory dz

significant toxicity

DO NOT USE w/Hep.B pts (increases viral replication)

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16
Q

Hep B management PAn

A

plasmapheresis to increase chance of seroconversion

antivirlas

17
Q

cause of death in PAN

A

uncontrolled vasculitis, infection complication due to tx induced immunosuppression, vascular complication

worse prognosis if pt has severe underlying dz

18
Q

systemic dz characterized by skin induration and thickening

A

scleroderma

+ chronic inflammatory infiltration in numerous visceral organs and vasculature

19
Q

scleroderma epidemiology

A

AFRICAN AMERICAN women

30-50

20
Q

pathogenesis of scleroderma

A
  1. overproduction of collagen and connective tissue proteins = fibrosis
  2. vasculitis of small vessels with obliteration
  3. abnormal activation of humoral and cellular immune systems
21
Q

scleroderma skin changes

A

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)

22
Q

pathophysiology of scleroderma

A

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

23
Q

categories of skin changes

A

limited cutaneous (limited to distal extremities)

diffuse cutaneous (skin thickening of turn, upper arms, thighs)

24
Q

non cutaneous symptoms of scleroderma

A
GI 
vascular 
pulmonary 
renal 
ENT 
CREST
25
Q

GI scleroderma

A

loss of LES = Gerd and aspiration

atrophy of peristaltic muscles = constipation, diarrhea, bacterial overgrowth and malnutrition

26
Q

vascular scleroderma

A

raynauds phenomenon (ALMOST ALL)

finger tip ulceration

27
Q

pulmonary scleroderma

A

pulmonary HTN

restrictive lung dz

28
Q

RENAL scleroderma

A

HTN
Renal iris
chronic renal insufficiency

29
Q

cardio scleroderma

A

CHF (myocardial fibrosis)
conduction system dz
syncope (arrhythmia)

30
Q

ENT scleroderma

A

SICCA
poor dentition, loss of teeth
hoarseness

31
Q

CREST syndrome

A

type of scleroderma

Calcinosis cutis (Ca deposits) 
Raynaud phenomenon 
Esophageal dysmotility
Sclerodactyly 
Telangioectasias
32
Q

renal crisis scleroderma

A

accelerated HTN, oliguria, HA, dyspnea, edema, rapidly rising serum Cr

diffuse, rapid skin involvement have highest risk (20-25%), BLACKS, MEN

33
Q

preventing renal crisis scleroderma

A

not treated = kidney failure

monitor BP and serum Cr

AVOID corticosteroids

34
Q

natural history scleroderma

A

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

35
Q

work up scleroderma

A
  1. ANA +
  2. topoisomerase I abs (absent in limited, + in diffuse)
  3. anti-centromere abs (absent in diffuse)
  4. PFTs and HRCT of chest
  5. Transthoracic echo/R Heart Cath for pulmonary HTN
  6. Esophageal studies
36
Q

tx of scleroderma

A

symptomatic management
Non-DHP CCB for raynauds
Meds for pulmonary HTN

37
Q

pregnancy scleroderma

A

high risk, esp if <4 yrs of diagnosis

38
Q

mortality scleroderma

A

12 yrs following diagnosis

poorer prognosis: young, rapid progression, lung/gi/cardiac involvement

pulmonary HTN prognostic indicator