Rheum D/O 3

Question 1

PAN

Answer 1

systemic vasculitis

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

Question 2

vasculitis of PAN results in

Answer 2

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

Question 3

what arteries does PAN like?

Answer 3

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

Question 4

PAN patho

Answer 4

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

Question 5

PAN spares…

Answer 5

LARGE vessels, SMALLEST vessels, venous system, pulmonary vasculature

Question 6

PAN MC affects

Answer 6

skin, joints, peripheral nerves, gut and kidney

Question 7

associated diseases

Answer 7

HEP B***

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

rheum dz

Question 8

PAN and Hep B

Answer 8

occur at any time during infection

MC

Question 9

onset of PAN

Answer 9

subacute

affects multiple organ systems

Question 10

PAN systems presentation (8)

Answer 10

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

Question 11

characteristic rash PAN

Answer 11

tender erythematous nodules with central ulcercation

Question 12

PAN work up

Answer 12

ESR/CRP elevated, negative AutoAbs

study of choice: angiography

confirm: tissue bx of cutaneous lesions, muscle, vessels

Question 13

tx of PAN

Answer 13

1. steroids
2. cyclophosphamide
3. management of underlying dz

Question 14

steroids and PAN

Answer 14

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

Question 15

cyclophosphamide and PAN

Answer 15

severe or steroid refractory dz

significant toxicity

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

Question 16

Hep B management PAn

Answer 16

plasmapheresis to increase chance of seroconversion

antivirlas

Question 17

cause of death in PAN

Answer 17

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

worse prognosis if pt has severe underlying dz

Question 18

systemic dz characterized by skin induration and thickening

Answer 18

scleroderma

+ chronic inflammatory infiltration in numerous visceral organs and vasculature

Question 19

scleroderma epidemiology

Answer 19

AFRICAN AMERICAN women

30-50

Question 20

pathogenesis of scleroderma

Answer 20

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

Question 21

scleroderma skin changes

Answer 21

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)

Question 22

pathophysiology of scleroderma

Answer 22

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

Question 23

categories of skin changes

Answer 23

limited cutaneous (limited to distal extremities)

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

Question 24

non cutaneous symptoms of scleroderma

Answer 24

GI 
vascular 
pulmonary 
renal 
ENT 
CREST

Question 25

GI scleroderma

Answer 25

loss of LES = Gerd and aspiration atrophy of peristaltic muscles = constipation, diarrhea, bacterial overgrowth and malnutrition

Question 26

vascular scleroderma

Answer 26

raynauds phenomenon (ALMOST ALL) finger tip ulceration

Question 27

pulmonary scleroderma

Answer 27

pulmonary HTN | restrictive lung dz

Question 28

RENAL scleroderma

Answer 28

HTN Renal iris chronic renal insufficiency

Question 29

cardio scleroderma

Answer 29

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

Question 30

ENT scleroderma

Answer 30

SICCA poor dentition, loss of teeth hoarseness

Question 31

CREST syndrome

Answer 31

type of scleroderma ``` Calcinosis cutis (Ca deposits) Raynaud phenomenon Esophageal dysmotility Sclerodactyly Telangioectasias ```

Question 32

renal crisis scleroderma

Answer 32

accelerated HTN, oliguria, HA, dyspnea, edema, rapidly rising serum Cr diffuse, rapid skin involvement have highest risk (20-25%), BLACKS, MEN

Question 33

preventing renal crisis scleroderma

Answer 33

not treated = kidney failure monitor BP and serum Cr AVOID corticosteroids

Question 34

natural history scleroderma

Answer 34

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

Question 35

work up scleroderma

Answer 35

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

Question 36

tx of scleroderma

Answer 36

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

Question 37

pregnancy scleroderma

Answer 37

high risk, esp if <4 yrs of diagnosis

Question 38

mortality scleroderma

Answer 38

12 yrs following diagnosis poorer prognosis: young, rapid progression, lung/gi/cardiac involvement pulmonary HTN prognostic indicator