Chap 186 Behcet Disease Flashcards

1
Q

____ is classified as a primary variable vessel vasculitis ,
emphasizing the involvement of any size and type (arterial, venous) of blood
vessel. It is also recognized as an autoinflammatory disease.

A

Behçet disease (BD)

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2
Q

Behçet disease has a high prevalence in countries along the ___

A

Silk Road

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3
Q

Onset in children is ___ yr of age

A

8-12

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4
Q

gene in which BD is markedly associated

A

HLA-B5101

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5
Q

A number of

infectious agents have been implicated and include ___

A

streptococci, herpes simplex

virus type 1, and parvovirus B19.

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6
Q

course of BD is characterized by

A

exacerbations and remissions

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7
Q

mean age of the first symptom is

A

between 8 and 12 yr

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8
Q

The most frequent

initial symptom is a

A

painful oral ulcer

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9
Q

description of oral ulcer of BD

A

oral ulcers are often
recurrent, may be single or multiple, range from 2-10 mm, and may be in anylocation in the oral cavity. They are often very painful. The oral ulcers last 3-10
days and heal without scarring

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10
Q

eye invlovment of BD

A

anterior uveitis, retinal vascultiis

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11
Q

skin lesions of BD range from

A

erythema nodosum (seen in approximately
50% of patients) to papulopustular acneiform lesions (85%), folliculitis, purpura,
and ulcers.

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12
Q

___ (seen in 50%) is another skin feature associated with BD
and is a pustular reaction occurring 24-48 hr after a sterile needle puncture or
saline injection; it is not pathognomonic of BD

A

Pathergy

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13
Q

____is the most common CNS manifestation in children

A

Dural sinus thrombosis

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14
Q

International Study Group for Behçet Disease (ISG) criteria are most
widely used and require the presence of

A
oral ulcers (at least 3 times per year)
along with 2 other major features, including genital ulcers, a positive pathergy
test, uveitis, and the characteristic skin lesions
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15
Q

is highly recommended to treat inflammatory eye disease

A

Azathioprine

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16
Q

For oral and genital ulcers, what topical

treatment is recommended

A

(sucralfate, corticosteroids)

17
Q

____ is
recommended for erythema nodosum or arthritis in males and females and for
genital ulcers in females

A

Colchicine

18
Q

For CNS disease and vasculitis, ____are recommended, and in

A

corticosteroids,

azathioprine, cyclophosphamide, and IFN-α

19
Q

unresponsive CNS disease, ___ is recommended

A

anti-TNF agents

20
Q

Mortality in children with BD is low except for the _____

A

pulmonary aneurysms