Behçet's syndrome is a rare autoinflammatory disease characterized by recurrent oral and genital ulcers, skin lesions, and eye inflammation. It is more common along the historic Silk Road with the highest prevalence in countries like Turkey, Iran, Japan and Korea. The cause is unknown but believed to involve a combination of genetic and environmental factors. Eye disease, in the form of retinal vasculitis and uveitis, is the most disabling manifestation and a leading cause of blindness. Treatment involves immunosuppressive medications and biologics targeting cytokines like TNF and IL-17.

1. Behçet’s syndrome
Marwa Abo Elmaaty Besar
Lecturer Of Internal Medicine
(Rheumatology Immunology Unit)
(Pediatric Rheumatology)

2.  Dr Hulusi Behcet (1889-1948).
 Triple symptom complex.
 Mediterranean basin, Korea, Japan- Silk road.
 Rare <puberty>50yr.
 Onset 20 years old.
 Male= female.
https://doi.org/10.1007/s10067-019-04570-w

3. Pathogenesis:
 Genetic vs Environmental :
• Japanese living in Hawaii
• Turkish immigrants vs Germans in Berlin
• Arabs/Druzes vs Jews in Israel
• North African immigrants vs Europeans in Paris
 “MHC-I-opathy”
• Mutations in ERAP1 gene,
• Important in functionality of Class I MHC loci, HLA-B51.
• Il-17/23 pathway is important for MHC-I-opathy
• IL-17 doesn’t work for Behcet but works for AS
• Apremilast works for Behcet, doesn’t work for AS.
 “Overlumping”
Tong et al.2019

5. Behçet’s
syndrome
Autoinflammatory
diseases
Adult
pediatric
Age group
affected
Silk Route
Mostly Europe
and
North America
Areas of highest
prevalence
No
yes
Monogenic
heritability
Very rare
Very common
Relapsing fever
Abates in time
Relentless unless
treated
Disease course

7. Oral ulcer Genital ulcer
Skin manifestation

8. Pathergy test
Path gnomic
Urate crystal test

9. Yazısız V. Behçet’s or Crohn’s disease?
Contrast-enhanced CT scan shows multiple polypoid lesions with a thickened colon wall (arrows).

10. Pulmonary
manifestation(PAA)
CNS
manifestation

11. Eye disease:
 Most serious.
 Leading cause of non traumatic blindness after DM in Japan, Israel.
 70% of male < 25 yr.
 At onset or first 2-3 yr.
 Bilateral in 90%.
 Rare after 5 yr.
 Retinal vasculitis, non granulomatous pan uveitis.
 Hypopyon 20%.

14. Hatemi G, et al. Ann Rheum Dis 2018;0:1–11. doi:10.1136/annrheumdis-2018-213225

15. Hatemi G, et al. Ann Rheum Dis 2018;0:1–11. doi:10.1136/annrheumdis-2018-213225

16.  The choice of immunomodulatory or immunosuppressive drug in such patients would depend
on individual patient characteristics regarding safety, the cost and availability of these agents
in each country, and patient preferences
 Dapsone and azithromycin.
 Interleukin (IL)-1 blockade with anakinra and canakinumab seems to provide a partial benefit
in BS patients.
 IL-6 blockade with tocilizumab worsened mucocutaneous lesions.
 IL-17 blockade with secukinumab was ineffective.
 IL12/23 blockade, ustekinumab may also be beneficial.
 lactobacilli lozenges may be a safe alternative.
 Leg ulcers, associated with pyoderma gangrenosum and require immunosuppressive.

17. Hatemi G, et al. Ann Rheum Dis 2018;0:1–11. doi:10.1136/annrheumdis-2018-213225

20. Hatemi G, et al. Ann Rheum Dis 2018;0:1–11. doi:10.1136/annrheumdis-2018-213225

21. Hatemi G, et al. Ann Rheum Dis 2018;0:1–11. doi:10.1136/annrheumdis-2018-213225

22. Hatemi G, et al. Ann Rheum Dis 2018;0:1–11. doi:10.1136/annrheumdis-2018-213225

24. Pearls :-
 Sacroiliitis and spinal involvement are not a feature of BS.
 Skin lesion do not include psoriasis.
 Urethral discharge is not a feature of BS.
 GI involvement with ileocecal ulceration, colonic perforation is distinct feature of IBD.
 Although multiple sclerosis shares the tendency of Behçet’s syndrome to involve the central nervous
system white matter, distinguishing these two conditions is usually straightforward.
 On neuroimaging, multiple sclerosis lesions tend to occur in a periventricular distribution, In
contrast, the typical brain lesions of Behçet’s syndrome have a subcortical location and extend into
the midbrain and brainstem.
 Both brainstem and cerebellar atrophy are often prominent in chronic behçet’s syndrome.

25. Reference:-
 Oğuz AK, Yılmaz ST, Oygür ÇŞ, et al. Behçet’s: a disease or a syndrome? Answer from an expression
profiling study. PLoS One. 2016;11e0149052.
 Kapsimali VD, Kanakis MA, Vaiopoulos GA, Kaklamanis PG. Etiopathogenesis of Behçet’s disease
with emphasis on the role of immunological aberrations. Clin Rheumatol. 2010;29:1211–1216.
 Mendes D, Correia M, Barbedo M, Vaio T, Mota M, Gonçalves O, et al.Behçet’s disease—a
contemporary review. J Autoimmun. (2009) 32:178–88.doi: 10.1016/j.jaut.2009.02.011.
 Criteria for diagnosis of Behçet’s disease. International Study Group for Behçet’s Disease. Lancet.
1990;335(8697):1078-80.
 Hamuryudan V, Melikoğlu M. Vascular Disease in Behçet’s Syndrome. 1st ed. In: Yazici Y, Yazici H,
editors. Behçet’s Syndrome (NY): Springer, 2010: 115-135.
 Radiologic and Clinical Findings of Behçet Disease: Comprehensive Review of Multisystemic
InvolvementEun Jin Chae et al., Radiographics, 2008