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Intestinal Behcet’s Disease (Enterobehcet)
Iraj Salehi-Abari Intestinal Behcet’s Disease (Enterobehcet) Iraj Salehi-Abari, MD. Internist, Rheumatologist Tehran, Iran
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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
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In the name of God the merciful and compassionate
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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Gross endoscopic picture of ITB:
Iraj Salehi-Abari Gross endoscopic picture of ITB: IntestinalTuberculosis (ITB): Annular ulcers along with cobblestone: Figure A y
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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
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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 onlyProbable EBD Typical ileocecal ulcer + None Suspected EBD Atypical ileocecal ulcer + Systemic BD Probable EBD Atypical ileocecal ulcer + Oral ulcer onlySuspected EBD Atypical ileocecal ulcer + None Nondiagnostic y
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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
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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
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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
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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
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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
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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
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