CTD: Sjogren, Scleroderma, DM. Lecture review Qs

Question 1

what is MAGIC syndrome and what are the antibodies against

Answer 1

behcet’s disease and relapsing polychondritis

antibodies against collagen type 2

Question 2

80% of patients with Sjogren syndrome have what antibody

Answer 2

anti-ro/SS-a

Question 3

what is the schirmer test

Answer 3

whatman paper applied to lower eyelid

migration <5mm over 5 min abnormal and indicates Sjogren syndrome

Question 4

what is the most definitive diagnostic tool for Sjogren

Answer 4

lower labial lip salivary gland biopsy

Question 5

what malignancy is associated with Sjogren syndrome

Answer 5

non-Hodgkin’s lymphoma

Question 6

what clinical features are seen in Sjogren syndrome

Answer 6

primary: xerostomia, keratoconjunctivitis sicca
secondary: autoimmune connective tissue disease

Question 7

stiff skin syndrome has been associated with what gene mutation

Answer 7

fibrillin-1

sparing of inguinal folds

Question 8

fibrillin-1 mutation is implicated in what two CTDs

Answer 8

stiff skin syndrome

systemic sclerosis

Question 9

in the new scoring system, what histiologic feature has the most points allocated for nephrogenic systemic fibrosis

Answer 9

osseous metaplasia

Question 10

nephrogenic systemic fibrosis is seen in what patients

Answer 10

pts on hemodialysis or pts recently exposed to gadalinium

Question 11

what is the most common antibody in mixed connective tissue disease

Answer 11

U1RNP

Question 12

what is sharp’s syndrome

Answer 12

mixed connective tissue diease

severe arthritis, hand edema (sausage digits), raynaud’s, esophageal dysmotility, pulmonary fibrosis, lymphadenopathy

Question 13

T/F: you have to see eosinophils on H&E for diagnosis of eosinophilic fasciitis

Answer 13

false

also only 10-40% of cases have peripheral eosinophilia

Question 14

groove sign, or dry river bed sign, is seen in what disease

Answer 14

eosinophilic fasciitis

Question 15

what is the major cause of death in systemic sclerosis

Answer 15

pulmonary ds

Question 16

what organ is most commonly involved in systemic sclerosis

Answer 16

esophagus (90%)

Question 17

what is the term for the distal nailbed adhering to the ventral nail plate in systemic sclerosis

Answer 17

pterygium inversum unguis

Question 18

what criteria is needed for the diagnosis of systemic sclerosis

Answer 18

1 major or 2 minor

major: proximal scleroderma to MCP joints
minor: sclerodactyly, digital pitting scars of fingertips, b/l basilar pulmonary fibrosis

Question 19

CREST syndrome antibodies

Answer 19

anti-centromere

Question 20

CREST is a form of localized systemic sclerosis. what are the features

Answer 20

Calcinosis, Raynauds, Esophageal dysmotility, Sclerodactyly, Telangiectasias

Question 21

what is Parry Romberg syndrome

Answer 21

progressive hemifacial atrophy
epilepsy
exopthalmos
alopecia

Question 22

antibodies in generalized morphea

Answer 22

anti-histone and ant-ssDNA

Question 23

anti-histone antibody diseases

Answer 23

generalized morphea

drug induced SLE

Question 24

anti-p155/140 is associated with

Answer 24

cancer in DM

see palmar involvement

Question 25

anti-CADM5 is associated with

Answer 25

interstitial lung disease in Japanese DM

Question 26

anti-Mi2 is associated with

Answer 26

good prognosis in DM

shawl sign, cuticular disease

Question 27

T/F: there is an increased risk of malignancy associated with childhood DM

Answer 27

false

increased incidence of calcinosis cutis

Question 28

the 2 types of childhood DM

Answer 28

Brunsting = slow course, progressive weakness, calcinosis. steroid responsive.
Banker = rapid onset, vasculitis of muscles and GI tract. steroid unresponsive, high mortality

Question 29

most common malignancies seen in DM (10-50% of cases)

Answer 29

women: ovarian
men: colon
asians: nasopharyngeal

Question 30

anti-Jo and other anti-synthetase antibodies (anti-PL7, anti-PL12, anti-DJ, anti-EJ) are associated with

Answer 30

interstitial lung ds in DM

Question 31

anti-Ku antibody is seen in what overlap syndrome

Answer 31

scleroderma and DM

Question 32

mechanic’s hands are associated with

Answer 32

anti-synthetase antibodies in DM.

indicate pulmonary involvement

Question 33

labs to check in DM

Answer 33

aldolase, CK
muscle biopsy (tricep, quad) and MRI
PFTs and CT scan if symptomatic

Question 34

most important clinical feature of DM

Answer 34

poikiloderma

Question 35

T/F: bullous DM is a good prognostic feature

Answer 35

false

indicates severe myopathy and lung ds

Question 36

in DM, skin findings usually precede muscle findings by how long

Answer 36

2-3 months

Question 37

age distribution seen in DM

Answer 37

bimodal

children (10-15 yrs) and adults (40-65 yrs)