Lupus and other CT disorders

Question 1

How many diagnostic criteria do you have meet to classify SLE?

Answer 1

4/11 criteria needs to be met

Question 2

how do you distinguish the butterfly (malar) rash from rosacea?

Answer 2

butterfly rash has a nasolabial food

Question 3

what are some symptoms of lupus?

Answer 3

• butterfly rash
• alopecia
• painless mucosal ulceration
• Jaccoud’s arthropathy
• CNS involvement

Question 4

what is the most devastating manifestation of lupus?

Answer 4

renal disease- lupus nephritis

Question 5

what is ANA testing useful for?

Answer 5

ANA has high sensitivity (100%) but low specificity

Question 6

what does (-) ANA tell you?

Answer 6

you do not have SLE

Question 7

what does (+) ANA tell you?

Answer 7

many causes for (+)ANA

• malignancies
• infections
• other autoimmune dz

Question 8

which test is SPECIFIC for SLE?

Answer 8

anti ds-DNA

Question 9

what is significant about (+)anti ds-DNA?

Answer 9

often associated with renal involvement (so it has poor prognosis)
-and it waxes and wanes with disease activity

Question 10

what other marker is quite specific for SLE?

Answer 10

anti-Smith

Question 11

which factors increase risk for avascular necrosis?

Answer 11

• steroid use
• antiphospholipid antibody positvity
• hyperlipidemia
• Raynaud’s phenomenon

Question 12

what is the hallmark of inflammatory myopathies?

Answer 12

painless proximal muscle weakness

Question 13

do children mostly have dermatomyositis or polymyositis?

Answer 13

children- dermatomyositis

Question 14

in adults, which inflammatory myopathy is more common?

Answer 14

polymyositis- most common

dermatomyositis may indicate paraneoiplastic syndrome

Question 15

what is Gottron’s papules?

Answer 15

rash around knucles indicative of dermatomyositis

Question 16

how many criteria do you need to fill for polymyositis?

Answer 16

4 out of 5 criteria

Question 17

how many criteria do yo uneed to fill for dermatoymyositis?

Answer 17

3 out of 4 criteria

Question 18

Name the 5 criteria for inflammatory myopathies.

Answer 18

• symmetric proximal muscle weakness
• elevated muscle enzymes
• myopathic EMG abnormalities
• typical changes on muscle biopsy
• typical rash of dermatomyositis

Question 19

which autoantibody is commonly seen in inflammatory myopathies?

Answer 19

anti-Jo1

Question 20

describe the involved skin in limited scleroderma.

Answer 20

distal extremities & face are involved (but trunk is spared)

Question 21

what does CREST stand for? CREST is a limited cutaneous system sclerosis.

Answer 21

Calcinosis
Raynaud's
Esopheal dysmotility
Sclerodactyly
Telangiectasias

Question 22

describe the color changes seen in Raynaud’s.

Answer 22

1st white- vasospasm
2nd blue- cyanosis
3rd red- reperfusion hyperemia

Question 23

which antibody is seen in limited scleroderma?

Answer 23

anti-centromere

Question 24

which antibody is seen in diffuse scleroderma?

Answer 24

anti-Scl-70

Question 25

describe treatment for scleroderma.

Answer 25

treatment is very frustrating-steroid use is limited; only symptomatic treatment

Question 26

what disease must be present for scleroderma?

Answer 26

Raynaud’s