1. Intestinal Behcet’s Disease (Enterobehcet)
Iraj Salehi-Abari
Intestinal Behcet’s Disease (Enterobehcet)
Iraj Salehi-Abari, MD.
Internist, Rheumatologist
Tehran, Iran

2. Iraj Salehi-Abari
Major sources: 1. Kelley & Firestein’s Textbook of Rheumatology; Curr Opin Rheumatol; Tadakazu et al, Diagnosis and management of intestinal Behcet’s disease. Clin J Gastroenterol; Farhad Shahram, The Book of Behcet’s Disease; 2000

3. In the name of God the merciful and compassionate

4. Iraj Salehi-Abari
Definition:
A Behcet’s Disease (BD) with intestinal involvement when there are:
. Typical ulcer(s) in ileocecal area and
. Systemic BD upon Classification Criteria y

5. Introduction: Entero-Behcet is one of the most dangerous types of BD
Iraj Salehi-Abari
Introduction:
Entero-Behcet is one of the most dangerous types of BD
Other dangerous types of BD are
Ocular-Behcet Disease
Neuro-Behcet Disease and
Vascular-Behcet Disease y

6. Epidemiology: The rate of Enterobehcet in BD is 0-60% (20%)
Iraj Salehi-Abari
Epidemiology:
The rate of Enterobehcet in BD is 0-60% (20%)
It is more common in East Asia
It is uncommon to rare in Mediterranean areas
It is more common in women:
F/M ratio > 1
But only in esophageal involvement; F/M ratio < 1 y

7. Iraj Salehi-Abari
Clinical feature:
The GI lesions in EBD are aphthous ulcers that resemble oral aphthosis
These can occur in all parts of GI tract but most commonly in:
Terminal ileum
Ileocecal region
Cecum and Colon y

8. Oral involvement: Oral aphthosis The most common feature
Iraj Salehi-Abari
Oral involvement:
Oral aphthosis
The most common feature
The cardinal manifestation and
The most common initial presentation
We know that mouth is a portion of GI tract, so oral aphthosis must be mentioned as a GI manifestation of BD. But in EBD it is excepted from GI manifestations. y

9. Other GI involvement: Pharyngeal involvement is uncommon
Iraj Salehi-Abari
Other GI involvement:
Pharyngeal involvement is uncommon
Esophageal involvement is uncommon
Gastroduodenal involvement is very rare
Small and Large intestinal involvement are the most common
Anorectal involvement is uncommon to rare y

10. Pharyngeal involvement:
Iraj Salehi-Abari
Pharyngeal involvement:
Pharyngeal aphthosis:
It can be detected through Laryngoscopy by ENT man
It is presented as Sore throat and Odynophagia y

11. Esophageal involvement:
Iraj Salehi-Abari
Esophageal involvement:
Esophageal aphthosis:
It can be presented as dysphagia, retrosternal pain and hematemesis due to:
Complications of aphthosis within esophagus eg, esophagitis, hemorrhagic esophagitis, esophageal perforation and stricture and rarely esophagotracheal fistula y

12. Esophageal involvement:
Iraj Salehi-Abari
Esophageal involvement:
Esophageal varices:
It is rare and its causes are:
Splenic vein thrombosis or
SVC thrombosis or
Suprahepatic vein thrombosis (Budd-Chiari Syndrome)
Above thrombosis are due to BD vasculitis + APS
It can be ruptured and presented as massive hematemesis or melena y

13. Gastroduodenal involvement:
Iraj Salehi-Abari
Gastroduodenal involvement:
Gastric &/or Duodenal aphthosis:
They are rare and their presentations are:
Dyspepsia
Epigastric pain
Nausea/Vomiting
Due to Gastroduodenitis or Peptic ulcer and
Rarely Pyloric stenosis y

14. Intestinal involvement:
Iraj Salehi-Abari
Intestinal involvement:
The intestinal features of Entero-Behcet Disease (EBD) are similar to Inflammatory Bowel Disease (IBD)
But in EBD; intestinal features usually appear months to years (usually years) after the onset of oral aphthosis
Whereas in IBD; intestinal features usually appear months to years before the onset of oral aphthosis y

15. Typical intestinal lesion in EBD:
Iraj Salehi-Abari
Typical intestinal lesion in EBD:
A giant oval-shaped deep punched-out ulcer in the ileocecal area y

16. Intestinal feature: Diffuse abdominal pain or pain in RLQ
Iraj Salehi-Abari
Intestinal feature:
Diffuse abdominal pain or pain in RLQ
Severe (+ bloody) diarrhea
Abdominal distension
Hematochezia or melena
Fever and weight loss y

17. Anorectal feature: Anorectal involvement is uncommon to rare
Iraj Salehi-Abari
Anorectal feature:
Anorectal involvement is uncommon to rare
Anal and/or rectal ( + aphthous) ulcers
Anal fissure and/or fistula
Rectovaginal fistula
Hemorrhagic proctitis y

18. Various types of EBD: Upon intestinal distribution:
Iraj Salehi-Abari
Various types of EBD:
Upon intestinal distribution:
Japanese type: ileocecal and right colon
Western type: left colon
Casabelanca type: both Rt. and Lt. colon y

19. Various types of EBD: Upon intestinal course: Mild type Wild type
Iraj Salehi-Abari
Various types of EBD:
Upon intestinal course:
Mild type
Wild type
The rate of mild type is 3 times more than wild type y

20. Mild type of EBD: Older ages Low titers of CRP Low DAIBD* (< 40)
Iraj Salehi-Abari
Mild type of EBD:
Older ages
Low titers of CRP
Low DAIBD* (< 40)
With remission after 8 weeks of treatment
Usually of Casabelanca type
*Disease Activity Index of Intestinal Behcet disease y

21. Wild type of EBD: Younger ages High titers of CRP High DAIBD (> 40)
Iraj Salehi-Abari
Wild type of EBD:
Younger ages
High titers of CRP
High DAIBD (> 40)
No remission after 8 weeks of treatment
Japanese type or Western type y

22. Differential Diagnosis of EBD:
Iraj Salehi-Abari
Differential Diagnosis of EBD:
Inflammatory Bowel Disease:
Crohn’s Disease: the most important DD
Ulcerative Colitis
Intestinal Tuberculosis
Infectious Enterocolitis y

23. The common extra-intestinal features of EBD and IBD:
Iraj Salehi-Abari
The common extra-intestinal features of EBD and IBD:
Oral aphthosis
Genital aphthosis
Ocular lesions
Erythema nodosum
Pustular lesion
Pyoderma gangrenosum
Arthritis y

24. The differences between ocular lesions of EBD and IBD:
Iraj Salehi-Abari
The differences between ocular lesions of EBD and IBD:
IBD ocular lesions:
Anterior Uveitis; usually acute/asymmetric (AAAU)
EBD ocular lesions:
Anterior Uveitis
Posterior (+ Intermediate) Uveitis
Retinal Vasculitis
Panuveitis y

25. Iraj Salehi-Abari
Clues in favour of EBD:
GI features occur months to years after oral aphthosis and may be other extra-GI features
Positive Pathergy test
Dominant extra-intestinal features
Bipolar (oral/genital) aphthosis
Skin aphthosis
Posterior Uveitis/Retinal Vasculitis
GI Pathology:
Absence of Granuloma and presence of Vasculitis y

26. Gross endoscopic pictures of EBD versus CD and ITB:
Iraj Salehi-Abari
Gross endoscopic pictures of EBD versus CD and ITB:
EBD: A few ( usually < 5) deep aphthous (Volcano) ulcers with skip area
Crohn’s Disease (CD): Multiple longitudinal Ulcers with cobblestone pattern of mucosa
IntestinalTuberculosis (ITB): Annular ulcers along with cobblestone y

27. Gross endoscopic picture of EBD:
Iraj Salehi-Abari
Gross endoscopic picture of EBD:
EBD: A few ( usually < 5) deep aphthous (Volcano) ulcers with skip area
Typical lesion:
A giant oval-shaped
deep punched-out ulcer
in the ileocecal area y

28. Gross endoscopic picture of CD:
Iraj Salehi-Abari
Gross endoscopic picture of CD:
Crohn’s Disease (CD): Multiple longitudinal Ulcers with cobblestone pattern of mucosa: Figure B y

29. Gross endoscopic picture of ITB:
Iraj Salehi-Abari
Gross endoscopic picture of ITB:
IntestinalTuberculosis (ITB): Annular ulcers along with cobblestone: Figure A y

30. Iraj Salehi-Abari
Complications of EBD:
The complications of Entero-Behcet Disease (EBD) are similar to Inflammatory Bowel Disease (IBD)
Perforation
Bleeding
Fistula
Obstruction
Abscess y

31. Patients with ulcer in ileocecal area:
Iraj Salehi-Abari
Patients with ulcer in ileocecal area:
Typical ileocecal ulcer + Systemic BD  Definite EBD
Typical ileocecal ulcer + Oral ulcer onlyProbable EBD
Typical ileocecal ulcer + None  Suspected EBD
Atypical ileocecal ulcer + Systemic BD Probable EBD
Atypical ileocecal ulcer + Oral ulcer onlySuspected EBD
Atypical ileocecal ulcer + None Nondiagnostic y

32. The Diagnosis of Behcet’s Disease
Iraj Salehi-Abari
The Diagnosis of Behcet’s Disease
International Study Group (ISG, 1990) criteria
. It is a classification criteria for research purposes
. It is useful for diagnosis of established Behcet’s Disease
. It is not a good instrument for early diagnosis of BD
International Criteria for Behcet’s Disease (ICBD, 2006)
. As same as above
2015 Persian Gulf Criteria (PGC, 2015) for Behcet’s D.
. The only criteria for early diagnosis of Behcet’s Disease y

33. Novel Diagnostic Criteria for intestinal Behcet’s disease (EBD)
Iraj Salehi-Abari
Novel Diagnostic Criteria for intestinal Behcet’s disease (EBD)
The patients with:
typical ileocecal ulcer and
Systemic BD
Have Definite intestinal Behcet’s disease (EBD) y

34. Treatment of EBD: Standard therapies: 5-ASA; Sulfasalazine
Iraj Salehi-Abari
Treatment of EBD:
Standard therapies:
5-ASA; Sulfasalazine
Corticosteroids
Immunomodulators: Azathioprine
Enteral nutrition
Total parenteral nutrition
Surgical therapy
Anti TNF-a therapy y

35. Treatment of EBD: Experimental therapies: Colchicine Thalidomide
Iraj Salehi-Abari
Treatment of EBD:
Experimental therapies:
Colchicine
Thalidomide
Antibiotics
Interferon
Combination therapy:
Endoscopic therapy with ethanol spraying &
Leukocytapheresis y

36. Treatment of EBD: Mild EBD: 5-ASA/Sulfasalazine (SSZ)
Iraj Salehi-Abari
Treatment of EBD:
Mild EBD: 5-ASA/Sulfasalazine (SSZ)
Remission: Maintenance SSZ
No response: SSZ + Steroids y

37. Treatment of EBD: Moderate to severe EBD: SSZ + Steroids:
Iraj Salehi-Abari
Treatment of EBD:
Moderate to severe EBD: SSZ + Steroids:
Remission: Maintenance SSZ
Steroid dependent or recurrent: + Azathioprine
Remission: Maintenance Azathioprine
No response: Surgery or Anti-TNFa y