22 - 141 - BEHCET DISEASE Flashcards
Behcet Disease activity has been to known to correlate with which bacterial infection?
a. Staphylococcus
b. Campylobacter
c. Pseudomonas
d. Streptococcus
D
What is the presenting sign in more than 80% of cases of Behcet Disease?
a. Oral aphthous ulcers
b. Genital ulcers
c. Arthritis/arthropathy
d. Pustular vasculitic lesions
A
What is the principal feature of pulmonary involvement in Behcet Disease?
a. Pulmonary Arterial Hypertension
b. Pulmonary Artery Aneurysm
c. Pulmonary Vein Obstruction
d. Pulmonary Embolism
B
What is NOT a MAJOR life threatening complication in patients with Behcet Disease?
a. CNS involvement
b. Pulmonary and large vessel involvement
c. Bowel Perforation
d. Deep vein thrombosis
D
Which medication can be used in pregnant patients with Behcet Disease?
a. Prednisolone
b. Dapsone
c. Cyclosporine
d. Azathioprine
A
A significant association exists between the Behcet disease and what HLA?
human leukocyte antigen-B 51(HLA-B51)
The allele also seems to be associated with a** more severe prognosis and ocular involvement.**
HLA-B51 alleles most frequently been associated with Adamantiades–Behçet disease.
HLA-B5101 and HLA-B5108
the DNa of this virus was detected in patients’ saliva and oral and genital ulcers
HSV-1
disease activity has been known to correlate with bacterial infection, particularly what spp?
Streptococci
the most relevant Streptococcus strain as a provoking factor for initiation of the disease
Streptococcus sanguinis
The major microscopic finding at most sites of active disease
immune-mediated occlusive vasculitis
have been detected as autoantigens in Adamantiades–Behçet disease
Tropomyosins and the 160-kDa polypeptide kinectin
sensitive marker of disease activity
IL-8
seems to play an important role, can also be released by endothelial cells, has a potent effect on the inflammatory response
most frequent clinical manifestations of Adamantiades–Behçet disease
Recurrent oral aphthous ulcers, recurrent genital ulcers, skin manifestations, ocular lesions, and arthritis/arthropathy
most frequently observed mucosal manifestations
Recurrent oral aphthous and genital ulcers
- Oral aphthous ulcers usually heal without scarring (92%). Genital ulcers may not recur as often and usually heal with a characteristic scar (64%-88%)
the presenting sign in more than 80% of the cases.
Oral aphthous ulcers
- Typically, lesions are multiple, painful, 1 to 3 cm in diameter, and sharply margined with a fibrin-coated base and surrounding erythema
- Oral aphthous ulcers usually heal without scarring (92%)
skin lesions of Adamantiades–Behçet disease
- pustular vasculitic lesions (including pathergy lesions),
- erythema nodosum-like lesions
- Sweet-like lesions
- pyoderma gangrenosum–like lesions,
- palpable purpuric lesions of necrotizing venulitis
- All of these lesions are characterized in their early stages by a neutrophilic vascular reaction.
- Single acneiform lesions or follicle-based pustules should not be considered relevant.
systemic manifestations of Adamantiades–Behçet disease
the major cause of morbidity in patients with Adamantiades–Behçet disease
Ocular involvement
The most diagnostically relevant lesion in ocular involvement in patients with Adamantiades–Behçet disease
posterior uveitis AKA retinal vasculitis
- which can lead to blindness
- Other ocular lesions include anterior uveitis, hypopyon (pus in the anterior chamber of the eye, which is now—due to early treatment—uncommon; see Fig. 141-6B), and secondary complications such as cataract, glaucoma, and neovascular lesions.
- Retinal inflammation can lead to vascular occlusion and, ultimately, tractional retinal detachment
The characteristic arthritis of patients with Adamantiades–Behçet disease
nonerosive, asymmetric, sterile, seronegative oligoarthritis
- symmetric polyarticular involvement is common.
- Joint manifestations frequently occur first in one knee or ankle and then the other as migratory monoarthritis, then in both joints simultaneously, and finally affecting nearly all joints
the principal feature of pulmonary involvement in Adamantiades–Behçet disease
Pulmonary artery aneurysms
- occasionally resulting in coughing and hemoptysis
clinical biomarkers of prognosis
Carotid plaques, pulse-wave velocity, and flow-mediated dilation
Characteristic histopathologic features of Adamantiades-Behçet diseas
vasculitis and thrombosis
- early mucocutaneous lesionsL show a neutrophilic vascular reaction with endothelial swelling, extravasation of erythrocytes, and leukocytoclasia or a fully developed leukocytoclastic vasculitis with fibrinoid necrosis of blood vessel walls
