141 - Amantiades-Behcet Disease

Question 1

Y/N: Amantiades-Behcet disease is classified as a systemic vasculitis involving _____ of blood vessels and characterized clinically by recurrent oral aphthous and genital ulcers, skin lesions, iridocylitis/posterior uveitis, and arthritis

Answer 1

All types and sizes

Question 2

Adamantiades-Behcet occurs worldwide with varying prevalence, being endemic in

Answer 2

Eastern and Central Asian

Eastern Mediterranean countries (along the so-called Silk Road)

Question 3

Adamantiades-Behcet disease most often affects patients in their

Answer 3

20s and 30s

Question 4

Y/N: Both genders are equally affected in Adamantiades-Behcet disease

Answer 4

Yes

Question 5

A significant association exists between Adamantiades-Behcet disease and HLA-_____ in Japan, the Middle East, and the Mediterranean countries

Answer 5

B51

Question 6

Viral agent detect in Adamantiades-Behcet disease patients’ saliva and oral and genital ulcers

Answer 6

HSV-1

Question 7

Dominates the flora of the oral mucosa in Adamantiades-Behcet disease patients with the disease and appears to be the most relevant strain as a provoking factor for initiation of the disease

Answer 7

Streptococcus sanguinis

Question 8

The lipoprotein of _____ (another bacterial agent) was found in the serum of patients with Adamantiades-Behcet disease but not in healthy controls

Answer 8

Mycoplasma fermentans MALP-404

Question 9

The pathergy reation is induced by the rapid accumulation of _____ (hyperchemotaxis) and later by _____ at needle prick sites

Answer 9

Neutrophils

T lymphocytes and monocytes/macrophages

Question 10

Adamantiades-Behcet disease has been considered to be a typical _____-mediated inflammatory disease, characterized by elevated levels of _____ cytokines

Answer 10

Th1

Question 11

Recently, some studies reported that _____-associated cytokines were increased; thus _____ cells and the _____ pathway may play important roles in the pathogenesis of Adamantiades-Behcet disease

Answer 11

Th17
Th17
IL17/IL23

Question 12

The _____ cells seem to be the primary target in the autoimmune mechanisms in Adamantiades-Behcet disease

Answer 12

Endothelial

Question 13

Most frequent clinical manifestations of Adamantiades-Behcet disease

Answer 13

Recurrent oral aphthous ulcers
Recurrent genital ulcers
Skin manifestations
Ocular lesions
Arthritis/arthropathy

Question 14

Most frequently observed mucosal manifestations in Adamantiades-Behcet disease

Answer 14

Recurrent oral aphthous and genital ulcers

Question 15

Presenting sign in more than 80% of cases of Adamantiades-Behcet disease

Answer 15

Oral aphthous ulcers

Question 16

Y/N: Oral aphthous ulcers in Adamantiades-Behcet disease usually heal with scarring (92%)

Answer 16

No - without

Question 17

Y/N: Genital ulcers in Adamantiades-Behcet disease may not recur as often and usually heal with a characteristic scar (64%-88%)

Answer 17

Yes

Question 18

Skin lesions that should be accepted as diagnostically relevant in Adamantiades-Behcet disease

Answer 18

Pustular vasculitic lesions
Erythema nodosum-like lesions
Sweet-like lesions
Pyoderma gangrenosum-like lesions
Palpable purpuric lesions of necrotizing venulitis

Question 19

Skin lesion that should not be considered relevant to Adamantiades-Behcet disease

Answer 19

Single acneiform lesions

Follicle-based pustules

Question 20

Major cause of morbidity in Adamantiades-Behcet disease

Answer 20

Ocular involvement

Question 21

Most diagnostically relevant ocular lesion in Adamantiades-Behcet disease

Answer 21

Posterior uveitis (called retinal vasculitis)

Question 22

Characteristic arthritis of Adamantiades-Behcet disease

Answer 22

Nonerosive, asymmetric, sterile, seronegative oligoarthritis

Question 23

Principal feature of pulmonary involvement in Adamantiades-Behcet disease

Answer 23

Pulmonary artery aneurysm

Question 24

GI involvement in Adamantiades-Behcet disease has an acute exacerbating course with ulcers, most commonly in the

Answer 24

Ileocolonic area

Question 25

Neurologic manifestations of Adamantiades-Behcet disease usually present with

Answer 25

Severe headache

Question 26

International Criteria for Behcet disease

Answer 26

Ocular lesions (recurrent) - 2
Oral aphthosis (recurrent) - 2
Genital aphthosis (recurrent) - 
Skin lesions (recurrent) - 1
CNS -1
Vascular manifestations - 1
Positive pathergy test - 1

Question 27

International Criteria for Behcet disease scoring which indicates Adamantiades-Behcet disease

Answer 27

More than or equal to 4

Question 28

Characteristic histopathologic features of Adamantiades-Behcet disease

Answer 28

Vasculitis and thrombosis

Question 29

Predominant histopathologic finding in Adamantiades-Behcet disease

Answer 29

Neutrophilic vascular reaction

Question 30

A positive pathergy test (hyperreactivity reaction) manifests within _____ as an erythematous papule (>_____mm) or pustule at the site of a skin needle prick or after intracutaneous injection of 0.1-mL isotonic saline using a 20-gauge needle without prior disinfection of the injection site

Answer 30

48 hours

2

Question 31

Y/N: Erythema without infiltration is considered a positive finding in the pathergy test

Answer 31

No - negative

Question 32

A positive pathergy reaction can also occur in patients with

Answer 32

Pyoderma gangrenosum
Rheumatoid arthritis
Crohn disease
Genital herpes infection

Question 33

Markers of severe prognosis in Adamantiades-Behcet disease

Answer 33

HLA-B51 positivity
Male gender
Early development of systemic signs