38 Behcet Disease Flashcards
Criteria for pediatric BD
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
Equal M:F, adult vs child
Child, adult men 2x as women
Strongest genetic assoc with BD
HLA B*5101
T/F 1 of the most characteristic features of BD is the heterogeneity of clinical presentation
T
Involvement of this organ system is increased in the clinical presentation of BD among ASIANS
GI
T/F Oral ulcerations in BD are usually painless
F, extremely painful
Oral ulcers of BD generally last for how long
3-10days
BD: HEal with scarring, oral vs genital ulcers
Genital ulcers
Unusual cutaneous pustular reaction occurring 24-48h after needle puncture of the dermis
Pathergy test
T/G Pathergy test is pathognomonic of BD
F, highly characteristic but NOT pathognomonic
Typical eye involvement of BD
Chronic relapsing bilateral anterior and posterior uveitis
MC CNS manif of BD in children
Increased ICP from cerebral venous sinus thrombosis
T/F Joint disease in BD does NOT usually result in erosions or joint deformities
T
The only vasculitis that involves both arterial and venous systems and can affect a vessel of any size
BD
Adult vs child BD: Superficial/deep venous thromboses
Adult
T/F GI lesiosn of BD are histologically indistinguishable from UC and Crohn’s
T
MC renal manif of BD
AMyloidosis and GN
T/F Cardiac manif are rare in BD
T
Prominent infiltrating cells in the synovium, skin, and eye lesions of BD
Neutrophils
Cytokine with a clear role in the pathogenesis of BD
IL-17, Highlights importance of IL-23/IL-17 pathway
Most important imaging studies in BD to delineate extent and character of vascular lesions
Angio
Most important imaging studies in BD to evaluate CNS effects of the diease
MRI
GI lesions of BD are most frequently where
Ileum
1st line treatment for oral and genital ulcers of BD
Topical sucralfate suspension or topical CS
Role of oral CS in BD
Fast relief during acute stage of ulceration
Option for severe BD ulceration
Thalidomide
Issues with thalidomide
High risk of peripheral neuropathy
Contraindicated in pregnancy
Other options for tx of ulcers in BD
Colchicine, anti-TNF esp Inflix
Treatment of ocular BD
AZA, systemic CS
REsistant: Add Cyclosporine or inflix, adalimumab
First-line for manif of BD other than ocular and ulcers
Prednisone
Steroid-sparing agent for GI BD
Sulfasalazine
Steroid-sparing agent for CNS BD
Aza, anti-tnf, CYC
T/F BD is usually monophasic
F, long relapsing course
Indicators of prolonged BD
Young age
Male
Organ system involvement in BD that can be severely incapacitating
Ocular and CNS
