38 Behcet Disease

Question 1

Criteria for pediatric BD

Answer 1

3/6
Recurrent oral aphthosis (3/year)
Genital ulceration typically with a scar
Skin features: Acneiform lesions, necrotic folliculitis, erythema nodosum
Ocular: Anterior/post uveitis, retinal vascultiits
Neuro signs, EXCEPT headache
Vascular: Venous/arterial thrombosis, arterial aneurysm

Question 2

Equal M:F, adult vs child

Answer 2

Child, adult men 2x as women

Question 3

Strongest genetic assoc with BD

Answer 3

HLA B*5101

Question 4

T/F 1 of the most characteristic features of BD is the heterogeneity of clinical presentation

Answer 4

T

Question 5

Involvement of this organ system is increased in the clinical presentation of BD among ASIANS

Answer 5

GI

Question 6

T/F Oral ulcerations in BD are usually painless

Answer 6

F, extremely painful

Question 7

Oral ulcers of BD generally last for how long

Answer 7

3-10days

Question 8

BD: HEal with scarring, oral vs genital ulcers

Answer 8

Genital ulcers

Question 9

Unusual cutaneous pustular reaction occurring 24-48h after needle puncture of the dermis

Answer 9

Pathergy test

Question 10

T/G Pathergy test is pathognomonic of BD

Answer 10

F, highly characteristic but NOT pathognomonic

Question 11

Typical eye involvement of BD

Answer 11

Chronic relapsing bilateral anterior and posterior uveitis

Question 12

MC CNS manif of BD in children

Answer 12

Increased ICP from cerebral venous sinus thrombosis

Question 13

T/F Joint disease in BD does NOT usually result in erosions or joint deformities

Answer 13

T

Question 14

The only vasculitis that involves both arterial and venous systems and can affect a vessel of any size

Answer 14

BD

Question 15

Adult vs child BD: Superficial/deep venous thromboses

Answer 15

Adult

Question 16

T/F GI lesiosn of BD are histologically indistinguishable from UC and Crohn’s

Answer 16

T

Question 17

MC renal manif of BD

Answer 17

AMyloidosis and GN

Question 18

T/F Cardiac manif are rare in BD

Answer 18

T

Question 19

Prominent infiltrating cells in the synovium, skin, and eye lesions of BD

Answer 19

Neutrophils

Question 20

Cytokine with a clear role in the pathogenesis of BD

Answer 20

IL-17, Highlights importance of IL-23/IL-17 pathway

Question 21

Most important imaging studies in BD to delineate extent and character of vascular lesions

Answer 21

Angio

Question 22

Most important imaging studies in BD to evaluate CNS effects of the diease

Answer 22

MRI

Question 23

GI lesions of BD are most frequently where

Answer 23

Ileum

Question 24

1st line treatment for oral and genital ulcers of BD

Answer 24

Topical sucralfate suspension or topical CS

Question 25

Role of oral CS in BD

Answer 25

Fast relief during acute stage of ulceration

Question 26

Option for severe BD ulceration

Answer 26

Thalidomide

Question 27

Issues with thalidomide

Answer 27

High risk of peripheral neuropathy
Contraindicated in pregnancy

Question 28

Other options for tx of ulcers in BD

Answer 28

Colchicine, anti-TNF esp Inflix

Question 29

Treatment of ocular BD

Answer 29

AZA, systemic CS
REsistant: Add Cyclosporine or inflix, adalimumab

Question 30

First-line for manif of BD other than ocular and ulcers

Answer 30

Prednisone

Question 31

Steroid-sparing agent for GI BD

Answer 31

Sulfasalazine

Question 32

Steroid-sparing agent for CNS BD

Answer 32

Aza, anti-tnf, CYC

Question 33

T/F BD is usually monophasic

Answer 33

F, long relapsing course

Question 34

Indicators of prolonged BD

Answer 34

Young age
Male

Question 35

Organ system involvement in BD that can be severely incapacitating

Answer 35

Ocular and CNS