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1
Background
Worldwide
Most prevalent in silk road
( Middle East, Mediterranean,…)
• Iraq: BD has been seen more frequently among
hospital patients in the last years.
• Behçet's disease is not uncommon in Iraq. Practising
physicians, dermatologists, and ophthalmologists must
be more aware of its existence.
Major mechanisms
Genetic predisposition : polygenic. include association
with certain human leukocyte antigens (HLA), including
HLA-B51, but both HLA and non-HLA genes may play a
role
Studies suggest a possible pathogenic role of certain
bacterial antigens that have crossreactivity with human
peptides. Different studies have implicated various
antigens and organisms, including streptococcal
antigens, Helicobacter pylori, Herpes simplex virus, and
parvovirus B19.
Direskeneli H. Behçet's disease: infectious aetiology, new autoantigens, and HLA-B51. Ann Rheum
7
Dis 2001; 60:996.
Altered innate immune function has been suggested by
some studies: deficiencies in mannose-binding lectin and
alterations in the expression of toll-like receptors.
Alterations in the numbers of T cell subpopulations and
evidence of cellular activation.
Humoral immune activation and autoantibodies have
been described against a number of targets. Immune
complexes may also play a role.
Direskeneli H. Behçet's disease: infectious aetiology, new autoantigens, and HLA-B51. Ann Rheum
Dis 2001; 60:996.
8
• Endothelial dysfunction and altered coagulation
:endothelial activation is a mediator of vascular
inflammation and thrombosis in BD.
• Neutrophil activation: exhibit increased motility and
enhanced adhesion to endothelial cells in vitro.
Direskeneli H. Behçet's disease: infectious aetiology, new autoantigens, and HLA-B51. Ann Rheum
Oral aphthae 2
Genital aphthae 2
Eye lesions 2
Skin lesions (PF, EN) 1
Vascular lesions 1
CNS lesions 1
Pathergy test 1 (some where)
Hatemi G, et al. EULAR recommendations for the management of Behçet disease. Ann Rheum Dis
2008; 67:1656. 22
There have been no randomized trials of infliximab in
Behçet’s disease.
However, efficacy in posterior uveitis has been
suggested by regimens in which infliximab is given at
a dose of 5 mg/kg at 0, 4, 8, 16, and 24 weeks or 0,
2, 6, and every 8 weeks. These regimens are similar
to that used for treatment of patients with moderately
to severely active Crohn's disease.
• Yamada Y et al.. Br J Ophthalmol 2010; 94:284.
• Giardina A, et al. Rheumatol Int 2011; 31:33.
23
Some patients require one or more
immunosuppressive agents indefinitely to sustain
remission.
Up to 50 percent of patients may be able to
completely discontinue immunosuppressive therapy
without recurrence of eye disease.
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