141 - Amantiades-Behcet Disease Flashcards
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
All types and sizes
Adamantiades-Behcet occurs worldwide with varying prevalence, being endemic in
Eastern and Central Asian
Eastern Mediterranean countries (along the so-called Silk Road)
Adamantiades-Behcet disease most often affects patients in their
20s and 30s
Y/N: Both genders are equally affected in Adamantiades-Behcet disease
Yes
A significant association exists between Adamantiades-Behcet disease and HLA-_____ in Japan, the Middle East, and the Mediterranean countries
B51
Viral agent detect in Adamantiades-Behcet disease patients’ saliva and oral and genital ulcers
HSV-1
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
Streptococcus sanguinis
The lipoprotein of _____ (another bacterial agent) was found in the serum of patients with Adamantiades-Behcet disease but not in healthy controls
Mycoplasma fermentans MALP-404
The pathergy reation is induced by the rapid accumulation of _____ (hyperchemotaxis) and later by _____ at needle prick sites
Neutrophils
T lymphocytes and monocytes/macrophages
Adamantiades-Behcet disease has been considered to be a typical _____-mediated inflammatory disease, characterized by elevated levels of _____ cytokines
Th1
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
Th17
Th17
IL17/IL23
The _____ cells seem to be the primary target in the autoimmune mechanisms in Adamantiades-Behcet disease
Endothelial
Most frequent clinical manifestations of Adamantiades-Behcet disease
Recurrent oral aphthous ulcers Recurrent genital ulcers Skin manifestations Ocular lesions Arthritis/arthropathy
Most frequently observed mucosal manifestations in Adamantiades-Behcet disease
Recurrent oral aphthous and genital ulcers
Presenting sign in more than 80% of cases of Adamantiades-Behcet disease
Oral aphthous ulcers
Y/N: Oral aphthous ulcers in Adamantiades-Behcet disease usually heal with scarring (92%)
No - without
Y/N: Genital ulcers in Adamantiades-Behcet disease may not recur as often and usually heal with a characteristic scar (64%-88%)
Yes
Skin lesions that should be accepted as diagnostically relevant in Adamantiades-Behcet disease
Pustular vasculitic lesions Erythema nodosum-like lesions Sweet-like lesions Pyoderma gangrenosum-like lesions Palpable purpuric lesions of necrotizing venulitis
Skin lesion that should not be considered relevant to Adamantiades-Behcet disease
Single acneiform lesions
Follicle-based pustules
Major cause of morbidity in Adamantiades-Behcet disease
Ocular involvement
Most diagnostically relevant ocular lesion in Adamantiades-Behcet disease
Posterior uveitis (called retinal vasculitis)
Characteristic arthritis of Adamantiades-Behcet disease
Nonerosive, asymmetric, sterile, seronegative oligoarthritis
Principal feature of pulmonary involvement in Adamantiades-Behcet disease
Pulmonary artery aneurysm
GI involvement in Adamantiades-Behcet disease has an acute exacerbating course with ulcers, most commonly in the
Ileocolonic area
Neurologic manifestations of Adamantiades-Behcet disease usually present with
Severe headache
International Criteria for Behcet disease
Ocular lesions (recurrent) - 2 Oral aphthosis (recurrent) - 2 Genital aphthosis (recurrent) - Skin lesions (recurrent) - 1 CNS -1 Vascular manifestations - 1 Positive pathergy test - 1
International Criteria for Behcet disease scoring which indicates Adamantiades-Behcet disease
More than or equal to 4
Characteristic histopathologic features of Adamantiades-Behcet disease
Vasculitis and thrombosis
Predominant histopathologic finding in Adamantiades-Behcet disease
Neutrophilic vascular reaction
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
48 hours
2
Y/N: Erythema without infiltration is considered a positive finding in the pathergy test
No - negative
A positive pathergy reaction can also occur in patients with
Pyoderma gangrenosum
Rheumatoid arthritis
Crohn disease
Genital herpes infection
Markers of severe prognosis in Adamantiades-Behcet disease
HLA-B51 positivity
Male gender
Early development of systemic signs
