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Patologie Immuni & Patologie Immuni &
Malattie OrfaneMalattie OrfaneTorino Torino –– 2222--24 Gennaio 200924 Gennaio 2009
Patologie Immuni & Patologie Immuni &
Malattie OrfaneMalattie OrfaneTorino Torino –– 2222--24 Gennaio 200924 Gennaio 2009
Entero BehçetEntero BehçetEntero BehçetEntero Behçet
PRESIDIO SANITARIO GRADENIGO PRESIDIO SANITARIO GRADENIGO PRESIDIO SANITARIO GRADENIGO PRESIDIO SANITARIO GRADENIGO PRESIDIO SANITARIO GRADENIGO PRESIDIO SANITARIO GRADENIGO PRESIDIO SANITARIO GRADENIGO PRESIDIO SANITARIO GRADENIGO TORINOTORINOTORINOTORINOTORINOTORINOTORINOTORINO
S.C. Gastroenterologia & EpatologiaS.C. Gastroenterologia & EpatologiaS.C. Gastroenterologia & EpatologiaS.C. Gastroenterologia & EpatologiaS.C. Gastroenterologia & EpatologiaS.C. Gastroenterologia & EpatologiaS.C. Gastroenterologia & EpatologiaS.C. Gastroenterologia & Epatologia
PRESIDIO SANITARIO GRADENIGO PRESIDIO SANITARIO GRADENIGO PRESIDIO SANITARIO GRADENIGO PRESIDIO SANITARIO GRADENIGO PRESIDIO SANITARIO GRADENIGO PRESIDIO SANITARIO GRADENIGO PRESIDIO SANITARIO GRADENIGO PRESIDIO SANITARIO GRADENIGO TORINOTORINOTORINOTORINOTORINOTORINOTORINOTORINO
S.C. Gastroenterologia & EpatologiaS.C. Gastroenterologia & EpatologiaS.C. Gastroenterologia & EpatologiaS.C. Gastroenterologia & EpatologiaS.C. Gastroenterologia & EpatologiaS.C. Gastroenterologia & EpatologiaS.C. Gastroenterologia & EpatologiaS.C. Gastroenterologia & Epatologia
F. RosinaF. RosinaF. RosinaF. RosinaF. RosinaF. RosinaF. RosinaF. Rosina
Entero BehçetEntero BehçetEntero BehçetEntero Behçet
Behçet disease Epidemiology of a rare disease
North America & North Europe 0,2 - 6,6 /100.000North America & North Europe 0,2 - 6,6 /100.000
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Behçet disease Epidemiology of a not so rare disease
North America & North Europe 0,2 - 6,6 /100.000North America & North Europe 0,2 - 6,6 /100.000North America & North Europe 0,2 - 6,6 /100.000
Far & Middle East 13,5 - 20/100.000
North America & North Europe 0,2 - 6,6 /100.000
Far & Middle East 13,5 - 20/100.000
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Behçet disease Epidemiology of a common disease
North America & North Europe 0,2 - 6,6 /100.000North America & North Europe 0,2 - 6,6 /100.000North America & North Europe 0,2 - 6,6 /100.000
Far & Middle East 13,5 - 20/100.000
Turkey 80 - 370 / 100,000
North America & North Europe 0,2 - 6,6 /100.000
Far & Middle East 13,5 - 20/100.000
Turkey 80 - 370 / 100,000
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Behçet disease Epidemiology of a common disease
North America & North Europe 0,2-6,6/100.000Far & Middle East 13,5-20/100.000Turkey 80-370 / 100,000
Age 20-40 yrs
North America & North Europe 0,2-6,6/100.000Far & Middle East 13,5-20/100.000Turkey 80-370 / 100,000
Age 20-40 yrs
Sex Men prevalence(Middle East)Female prevalence(Far East & North Europe)
Severity More severe in Young MenMiddle & Far East
Sex Men prevalence(Middle East)Female prevalence(Far East & North Europe)
Severity More severe in Young MenMiddle & Far East
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Behçet disease Definition
Behcet's disease is a chronic, relapsing vasculitis involving arterial and venous blood vessels of all sizes
Behcet's disease is a chronic, relapsing vasculitis involving arterial and venous blood vessels of all sizesand venous blood vessels of all sizesleading to several systemic manifestations….
and venous blood vessels of all sizesleading to several systemic manifestations….
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Behçet disease Clinical manifestationsBehçet disease Clinical manifestations
100
75 75 75
60
70
80
90
100
50
33
20
1 1 1
10
0
10
20
30
40
50
60
Oral U
lcers
Uroge
nital
SkinArth
ritis
EyeVas
cular
Neura
l
Renal
Cardi
ac
Lung GI
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Behçet disease Large Vessels Disease
Number of
patients
Arterial disease
Pulmonary artery occlusion or aneurysm 36/728 (5%)
Aortic aneurysm 17/728 (2%)
Extremity arterial occlusion or aneurysm 45/728 (6%)
Other arterial occlusion or aneurysm 42/728 (6%)
Right ventricular thrombus 2/728
Review of 728 cases from Koc, Y, Gullu, I, Akpek, G, et al. J Rheumatol 1992; 19:402.
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Behçet disease Enteral Arterial disease
• Massive gastrointestinal bleeding due to aneurysmal
rupture of ileo-colic artery. After the failure of hemostasis
with arterial embolization, ileo-right colecctomy wa s performed.
(Kim SU et al Korean J Gastroenterol. 2007) (Kim SU et al Korean J Gastroenterol. 2007)
• Massive hemorrhage from ruptured small aneurysm on
the right ileocolic artery successfully treated with
superselective arterial embolization using microcoi ls (Hong
YK, Yoo WH. Rheumatol Int 2008)
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Behçet disease Large Vessels Disease
Number of patients
Venous diseaseDeep venous thrombosis 221/728 (30%)
Subcutaneous thrombophlebitis 205/728 (28%)Subcutaneous thrombophlebitis 205/728 (28%)
SVC occlusion 122/728 (17%)
IVC occlusion 93/728 (13%)
Cerebral sinus thrombosis 30/728 (4%)
Budd-Chiari syndrome 17/728 (2%)
Other venous occlusion 24/728 (3%)
Review of 728 cases from Koc, Y, Gullu, I, Akpek, G, et al. J Rheumatol 1992; 19:402.
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Behçet disease Large vessel Disease
Number of patients
Hepatic Vein Thrombosis 14/493 (3%)
Hepatic Vein (HV) Thrombosis Alone 4/14 (29%)
HV + Inferior Vena Cava (IVC) Thrombosis 8/14 (57%)
HV + IVC + Portal Vein Thrombosis 2/14 (16%)
Bayraktar Y et al.. Am J Gastroenterol 1997
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Behçet disease Budd Chiari
• Acute — 20 % (5% with fulminant hepatic failure)
• Subacute — 40 % (signs or symptoms for less than six months and no evidence of liver cirrhosis)
• Chronic — 40 % (signs or symptoms for more than six months with evidence of portal hypertension and cirrhosis)
• Acute — 20 % (5% with fulminant hepatic failure)
• Subacute — 40 % (signs or symptoms for less than six months and no evidence of liver cirrhosis)
• Chronic — 40 % (signs or symptoms for more than six months with evidence of portal hypertension and cirrhosis)
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
BehçetSmall Vessels Disease: Entero Behçet
Symptoms • anorexia• nausea• abdominal pain • diarrhea
Symptoms • anorexia• nausea• abdominal pain • diarrhea
Lesions• “punched out” or “flask shaped” ulcers involving both the submucosa and the muscularis propria, especially in the ileo-ciecal region, less frequenltly in the esophagus and rarely in the stomach and duodenum
Lesions• “punched out” or “flask shaped” ulcers involving both the submucosa and the muscularis propria, especially in the ileo-ciecal region, less frequenltly in the esophagus and rarely in the stomach and duodenum
Griffin JW et al. South Med J 1982Anti M et al. J Clin Gastroenterol 1986Powderly WG et al. Ir J Med Sci 1987Stringer DA et al. Pediatr Radiol 198
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Behçet disease Clinical manifestations
ItalyItaly TurkeyTurkey ChinaChina TaiwanTaiwan KoreaKorea ThaiThai
Number of studied ptsNumber of studied pts 137137 23132313 2828 125125 15271527 2323
•• Oral ulcerationsOral ulcerations 99%99% 100%100% 95%95% NRNR 99%99% 100%100%
•• UroUro--genital lesionsgenital lesions 63%63% 85%85% 50%50% NRNR 83%83% 70%70%
•• Cutaneous lesionsCutaneous lesions 82%82% 80%80% 95%95% NRNR 84%84% 61%61%
•• Ocular diseaseOcular disease 61%61% 38%38% 50%50% NRNR 51%51% 52%52%
•• Neurologic diseaseNeurologic disease NRNR 3%3% NRNR NRNR NRNR 9%9%
ItalyItaly TurkeyTurkey ChinaChina TaiwanTaiwan KoreaKorea ThaiThai
Number of studied ptsNumber of studied pts 137137 23132313 2828 125125 15271527 2323
•• Oral ulcerationsOral ulcerations 99%99% 100%100% 95%95% NRNR 99%99% 100%100%
•• UroUro--genital lesionsgenital lesions 63%63% 85%85% 50%50% NRNR 83%83% 70%70%
•• Cutaneous lesionsCutaneous lesions 82%82% 80%80% 95%95% NRNR 84%84% 61%61%
•• Ocular diseaseOcular disease 61%61% 38%38% 50%50% NRNR 51%51% 52%52%
•• Neurologic diseaseNeurologic disease NRNR 3%3% NRNR NRNR NRNR 9%9%
•• Vascular diseaseVascular disease NRNR 25%25% NRNR NRNR NRNR 9%9%
•• ArthritisArthritis NRNR 11%11% 45%45% NRNR NRNR NRNR
•• Renal diseaseRenal disease NRNR NRNR NRNR NRNR NRNR NRNR
•• Cardiac diseaseCardiac disease NRNR NRNR NRNR NRNR NRNR NRNR
•• Pulmonary diseasePulmonary disease NRNR 0,03%0,03% NRNR NRNR NRNR NRNR
•• Vascular diseaseVascular disease NRNR 25%25% NRNR NRNR NRNR 9%9%
•• ArthritisArthritis NRNR 11%11% 45%45% NRNR NRNR NRNR
•• Renal diseaseRenal disease NRNR NRNR NRNR NRNR NRNR NRNR
•• Cardiac diseaseCardiac disease NRNR NRNR NRNR NRNR NRNR NRNR
•• Pulmonary diseasePulmonary disease NRNR 0,03%0,03% NRNR NRNR NRNR NRNR
Salvarani C et al. Arthritis Rheum 2007Pipitone N et al. Clin Exp Rheumatol 2004
Tursen U et al. Int J Dermatol 2003Ning Sheng L et al. Clin Rheumatol 2005Chou SJ et al J Gastrointest Surg 2007
Ling J et al Yan Ke Xue Bao 2005Bang D et al. J Korean Med Sci 2001
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Behçet disease Gastrointestinal
Behçet disease Gastrointestinal
66
60
70
80
90
100* 43% HP/NSAIDs neg.ve Peptic ulcers
0 1,5
9 9
35
0
10
20
30
40
50
60
Italy
Turke
y
Korea
Thai
China*
Taiwan
Salvarani C et al. Arthritis Rheum 2007Pipitone N et al. Clin Exp Rheumatol 2004
Tursen U et al. Int J Dermatol 2003Ning Sheng L et al. Clin Rheumatol 2005Chou SJ et al J Gastrointest Surg 2007
Ling J et al Yan Ke Xue Bao 2005Bang D et al. J Korean Med Sci 2001
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Entero BehçetNatural History
Remitting relapsing disease complicated by Intestinal perforation
Taiwan125 Behçet’s disease cases Follow-up 25 yrsFollow-up 25 yrs82/125 (66%) intestinal Behçet’s disease 22/82 (27%) intestinal perforationMale/female 14/8 - Age 22-65 yrsSurgery:• Hemicolectomy 11• Partial Ileum Resection 8• Ileocecal Resection 3Reperforation:3
Chou SJ et al J Gastrointest Surg 2007Choi IJ et al. Dis Colon Rectum 2000
Kin Js et al. Endoscopy 2000
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Entero BehçetTreatment
Glucocorticoids plus Azathiopine / 6-mercaptopurine• Prednisone 0.5 to 1.0 mg/kg daily• Azathioprine 2.5 – 2.5 mg/k / daily
Infliximab3 to 5 mg/kg at 0, 2, and 6 weeks, followed by 5 mg/kg every 8 weeks3 to 5 mg/kg at 0, 2, and 6 weeks, followed by 5 mg/kg every 8 weeks(Travis SP et al , Gut 2001; Kram MT et al, Dis Colon Rectum. 2003; Naganuma M et al, Inflamm Bowel Dis 2008; Lee JH et al Korean J Intern Med 2007))
Thalidomide 2 mg/kg per day, (Yasui K et al, J Pediatr 2003; Yasui K et al Inflamm Bowel Dis 2008)
Cyclosporin
Surgery
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Entero BehçetDiagnosis
……the problem is not to assess the presence of GI involvement in a patient with a known Behçet disease, but ……
……the problem is not to assess the presence of GI involvement in a patient with a known Behçet disease, but …… a known Behçet disease, but ……
to make a diagnosis of Behçet syndrome in a patient with GI ulcers
a known Behçet disease, but ……
to make a diagnosis of Behçet syndrome in a patient with GI ulcers
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Entero BehçetDiagnosis
• Radiology
• Endoscopy• Upper GI Endoscopy• Colonoscopy
Wireless Capsule Endoscopy
• Radiology
• Endoscopy• Upper GI Endoscopy• Colonoscopy
Wireless Capsule Endoscopy• Colonoscopy• Wireless Capsule Endoscopy
• Histology
• Serology
but ……
• Colonoscopy• Wireless Capsule Endoscopy
• Histology
• Serology
but ……
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Entero BehçetDiagnosis
Small Bowel & Colon Barium Studies• mucosal fold thickening
• discrete ulcers • poor sensitivity and specificity
Small Bowel & Colon Barium Studies• mucosal fold thickening
• discrete ulcers • poor sensitivity and specificity
CT scans• bowel wall thickening• perienteric infiltration • polipoid bowel involvement• polipod mass-like lesions
CT scans• bowel wall thickening• perienteric infiltration • polipoid bowel involvement• polipod mass-like lesions
Chung SY et alRadiographics 2001
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Entero BehçetDiagnosis
• Endoscopy• Upper GI Endoscopy• Colonoscopy• Small Bowel Capsule Endoscopy
• Endoscopy• Upper GI Endoscopy• Colonoscopy• Small Bowel Capsule Endoscopy
Crohn’s
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Behçet’s
Entero BehçetWireless Capsule Endoscopy
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Thomson M et al. J Pediatr Gastroenterol Nutr 2007Hamdulay SS et al Rheumatology Oxford 2008
Barium-CT pos.ve / suspected Behçet (0/11 - 0%)
WCE pos.ve / suspected Behçet ( 10/11 - 91%)
Barium-CT pos.ve / suspected Behçet (0/11 - 0%)
WCE pos.ve / suspected Behçet ( 10/11 - 91%)
Entero BehçetDiagnosis
• Endoscopy• Upper GI Endoscopy• Colonoscopy• Small Bowel Capsule Endoscopy
• Histology
• Endoscopy• Upper GI Endoscopy• Colonoscopy• Small Bowel Capsule Endoscopy
• Histology • Histology does not discriminate
between Behçet and other Gastrointestinal Vasculitis, specific for IBD only when early lesions are present
• Serology
• Histology does not discriminate
between Behçet and other Gastrointestinal Vasculitis, specific for IBD only when early lesions are present
• Serology
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Entero BehçetDiagnosis
Serology
ASCA IgA & IgG
Crohn Disease 42%Behçet Disease 4%
Serology
ASCA IgA & IgG
Crohn Disease 42%Behçet Disease 4%Behçet Disease 4%Ulcerative Colitis 4%Ankilosing Spondilitis 15%
Behçet Disease 4%Ulcerative Colitis 4%Ankilosing Spondilitis 15%
Fresko I et al. Clin Exper Rheumatol 2005Choi CH et al. Dis Colon Rectum 2006
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Entero BehçetDiagnosis
Serology
ASCA IgA & IgG
Crohn Disease 42%Behçet Disease 4%
Serology
ASCA IgA & IgG
Crohn Disease 42%Behçet Disease 4%Behçet Disease 4%Ulcerative Colitis 4%Ankilosing Spondilitis 15%Enteral Behçet 44%
……..severity and relapse rates of intestinal Behçet's disease not associated with ASCA expression. ASCA pos.ve pts more likely to receive surgical treatment.
Behçet Disease 4%Ulcerative Colitis 4%Ankilosing Spondilitis 15%Enteral Behçet 44%
……..severity and relapse rates of intestinal Behçet's disease not associated with ASCA expression. ASCA pos.ve pts more likely to receive surgical treatment.
Fresko I et al. Clin Exper Rheumatol 2005Choi CH et al. Dis Colon Rectum 2006
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Entero BehçetDiagnosis
• Barium Studies
• Endoscopy• Upper GI Endoscopy• Colonoscopy• Small Bowel Capsule Endoscopy
• Barium Studies
• Endoscopy• Upper GI Endoscopy• Colonoscopy• Small Bowel Capsule Endoscopy• Colonoscopy• Small Bowel Capsule Endoscopy
• Biopsy
• Serology
• International Diagnostic Criteria
• Colonoscopy• Small Bowel Capsule Endoscopy
• Biopsy
• Serology
• International Diagnostic Criteria
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Entero BehçetInternational Diagnostic Criteria
Recurrent Oral Aphthae (three times in one year)
plus two of the following in the absence of other systemic diseases:
• Recurrent genital aphthae
• Eye lesions (including anterior or posterior uveitis, cells in vitreous on slit
Recurrent Oral Aphthae (three times in one year)
plus two of the following in the absence of other systemic diseases:
• Recurrent genital aphthae
• Eye lesions (including anterior or posterior uveitis, cells in vitreous on slit • Eye lesions (including anterior or posterior uveitis, cells in vitreous on slit
lamp examination, or retinal vasculitis, observed by an ophthalmologist)
• Skin lesions (including erythema nodosum, pseudo vasculitis, papulopustular
lesions, or acneiform nodules consistent with Behcet's)
• A positive pathergy test (a papule 2 mm or more in size developing 24 to
48 hours after oblique insertion of a 20 to 25 gauge needle 5mm into the skin,
generally performed on the forearm)
• Eye lesions (including anterior or posterior uveitis, cells in vitreous on slit
lamp examination, or retinal vasculitis, observed by an ophthalmologist)
• Skin lesions (including erythema nodosum, pseudo vasculitis, papulopustular
lesions, or acneiform nodules consistent with Behcet's)
• A positive pathergy test (a papule 2 mm or more in size developing 24 to
48 hours after oblique insertion of a 20 to 25 gauge needle 5mm into the skin,
generally performed on the forearm)
International Study Group for Behcet's Disease. Lancet 1990
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Entero BehçetSensitivity & specificity of Behçet Diagnostic criteria
Sensitivity Specificity
International 95% 100%
Sensitivity Specificity
International 95% 100%International 95% 100%
Japanese 100% 95%
Mason & Barnes 100% 94%
International 95% 100%
Japanese 100% 95%
Mason & Barnes 100% 94%
International Study Group for Behcet's Disease. Lancet 199Tunc R etr al. Clin Exp Rheumatol 2001
Chang HK et al Clin Exp Rheumatol 2004
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Entero BehçetInternational Diagnostic Criteria
Recurrent Oral Aphthae (three times in one year)
plus two of the following in the absence of other systemic diseases:
• Recurrent genital aphthae
• Eye lesions (including anterior or posterior Uveitis , cells in vitreous on slit
Recurrent Oral Aphthae (three times in one year)
plus two of the following in the absence of other systemic diseases:
• Recurrent genital aphthae
• Eye lesions (including anterior or posterior Uveitis , cells in vitreous on slit • Eye lesions (including anterior or posterior Uveitis , cells in vitreous on slit
lamp examination, or retinal vasculitis, observed by an ophthalmologist)
• Skin lesions (including Erythema Nodosum , pseudo vasculitis,
papulopustular lesions, or acneiform nodules consistent with Behcet's)
• A positive pathergy test (a papule 2 mm or more in size developing 24 to
48 hours after oblique insertion of a 20 to 25 gauge needle 5mm into the skin,
generally performed on the forearm)
• Eye lesions (including anterior or posterior Uveitis , cells in vitreous on slit
lamp examination, or retinal vasculitis, observed by an ophthalmologist)
• Skin lesions (including Erythema Nodosum , pseudo vasculitis,
papulopustular lesions, or acneiform nodules consistent with Behcet's)
• A positive pathergy test (a papule 2 mm or more in size developing 24 to
48 hours after oblique insertion of a 20 to 25 gauge needle 5mm into the skin,
generally performed on the forearm)
International Study Group for Behcet's Disease. Lancet 1990
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Entero BehçetSensitivity & specificity of Behçet Diagnostic criteria
Sensitivity Specificity
International 95% 100%
Japanese 100% 95%
Sensitivity Specificity
International 95% 100%
Japanese 100% 95%
Mason & Barnes 100% 94%
… inclusion of pts with Crohn disease and Ulcerative Colitis did not
substantially change sensitivity & specificity of ISG (International Study
Group) diagnostic score
Mason & Barnes 100% 94%
… inclusion of pts with Crohn disease and Ulcerative Colitis did not
substantially change sensitivity & specificity of ISG (International Study
Group) diagnostic score
International Study Group for Behcet's Disease. Lancet 199Tunc R etr al. Clin Exp Rheumatol 2001
Chang HK et al Clin Exp Rheumatol 2004
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Entero BehçetConclusions
.....if you have still some doubt on your diagnosis……......if you have still some doubt on your diagnosis…….
…. just rule out infective colitis and feel comfortable since …….…. just rule out infective colitis and feel comfortable since …….
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Entero BehçetConclusions
Steroids
Azathioprine
Steroids
Azathioprine
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
InfliximabInfliximab
Entero BehçetConclusions
Steroids
Azathioprine
Steroids
Azathioprine
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Infliximab
…… are perfectly fitting with both Behçet’ and Crohn’s diseases
Infliximab
…… are perfectly fitting with both Behçet’ and Crohn’s diseases
Entero BehçetConclusions
Crohn’s Behçet’s
Steroids OK OK
Crohn’s Behçet’s
Steroids OK OK
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
Azathioprine OK OK
Infliximab OK OK
Azathioprine OK OK
Infliximab OK OK
…if you still have some doubt on the diagnosis,
Just rule out infective colitis and fell confortable since …….
Crohn’s Behçet’sCrohn’s Behçet’s
Steroids OK OK
Azathioprine OK OK
Infliximab OK OK
….. Perfectly fitting for both Behçet’s and Crohn’s disease.
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
The Mason–Barnes Criteria
Major Symptoms Minor Symptoms
Buccal ulcerations Gastrointestinal lesions
Genital ulcerations Thrombophlebitis
Ocular lesions Cardiovascular lesionsOcular lesions Cardiovascular lesions
Skin lesions
Arthritis
Neurologic lesions
Family history
Three major or two major and two minor criteria are required to establish the diagnosis of Behçet’s disease
Entero BehçetSensitivity & specificity of Behçet Diagnostic criteria
Sensitivity Specificity
International 95% 100%
Sensitivity Specificity
International 95% 100%International 95% 100%
Japanese (Suzuki) 100% 95%
Mason & Barnes 100% 94%
International 95% 100%
Japanese (Suzuki) 100% 95%
Mason & Barnes 100% 94%
International Study Group for Behcet's Disease. Lancet 199Tunc R etr al. Clin Exp Rheumatol 2001
Chang HK et al Clin Exp Rheumatol 2004
12° Convegno Patologie Immuni & Malattie Orfane , Torino 22-24 gennaio 2009
XIII Congresso Nazionale delle XIII Congresso Nazionale delle
Malattie DigestiveMalattie Digestive
Corso AIGOCorso AIGO--SIGESIGE
“Hot topics in Gastroenterologia”“Hot topics in Gastroenterologia”Palermo Palermo –– 3 ottobre 20073 ottobre 2007
XIII Congresso Nazionale delle XIII Congresso Nazionale delle
Malattie DigestiveMalattie Digestive
Corso AIGOCorso AIGO--SIGESIGE
“Hot topics in Gastroenterologia”“Hot topics in Gastroenterologia”Palermo Palermo –– 3 ottobre 20073 ottobre 2007
“Up date” su …. Colangite Sclerosante “Up date” su …. Colangite Sclerosante Colangite Sclerosante PrimitivaColangite Sclerosante Primitiva
F. RosinaPresidio Sanitario GradenigoTorino
F. RosinaPresidio Sanitario GradenigoTorino
Behçet disease Clinical manifestations
• Oral ulcerations (95-100%)• Uro-genital lesions (75%)• Cutaneous lesions (>75%)• Ocular disease (25-75%)• Neurologic disease ( < 20%)• Vascular disease (33%)• Arthritis (75%)• Renal disease (uncommon)• Cardiac disease (uncommon)• Pulmonary diasease (uncommon)• Gastrointestinal Symptoms (common)
20° Congresso Regionale ACOI , 31 ottobre 2008
• Gastrointestinal Symptoms (common)• Gastrointestinal disease (more or less frequent depending on the geographical area: unfrequent in in Italy and middle east – common in far east (Japan, Korea)•Severity is generally greater in men. The greatest morbidity and mortality occurs with ocular disease (affecting up to two-thirds of patients), vascular disease (affecting up to one-third of patients), and central nervous system disease (affecting 10 to 20 percent of patients). Cutaneous and articular manifestations are common. Renal disease and peripheral nervous system involvement are less common than in other vasculitides
Behçet disease Epidemiology
Behcet's disease is more common (and often more severe) along the ancient silk road, which extends from eastern Asia to the Mediterranean [4]. It is most common in Turkey (80 to 370 cases per 100,000) while the prevalence ranges from 13.5 to 20 per 100,000 in Japan, Korea, China, Iran, Iraq, and Saudi Arabia [4]. By comparison, the prevalence is from 1:15,000 to 1:500,000 in Northern American (Olmsted County, Minnesota) and Northern European countries. It is somewhat more common in men in the eastern Mediterranean area and in women in north
20° Congresso Regionale ACOI , 31 ottobre 2008
more common in men in the eastern Mediterranean area and in women in north European countries, and typically affects young adults 20 to 40 years of age. The disease appears to be more severe in young, male, and Middle Eastern or Far Eastern patients
FARE TABELLA IMPOSTANDOLA TUTTA SU UN RAPPORTO …./100.000
clinical manifestations vary among different patients and populations. Men and women have different propensities to develop the disease, but this varies among populations. As an example, Behcet's is more common in men in the Middle East and in women in Japan and Korea [6].
Behçet disease Vascular disease
Most clinical manifestations of Behcet's disease are due to vasculitis involving:
Most clinical manifestations of Behcet's disease are due to vasculitis involving:
20° Congresso Regionale ACOI , 31 ottobre 2008
vasculitis involving:
• blood vessels of all sizes
• arterial and venous
vasculitis involving:
• blood vessels of all sizes
• arterial and venous
Behçet disease Budd Chiari etiology Myeloproliferative diseases
Malignancy
Hepatocellular carcinoma
Infections and benign lesions of the liver
Oral contraceptives
Pregnancy
Hypercoagulable states
20° Congresso Regionale ACOI , 31 ottobre 2008
Hypercoagulable states(Factor V Leiden mutation, Prothrombin gene mutation, Antiphospholipid antibody syndrome, Antithrombin III deficiency, Protein C deficiency, Protein S deficiency )
Paroxysmal nocturnal hemoglobinuria
JAK2 mutations
Behcet's disease
Membranous webs of the inferior vena cava and/or the hepatic veins
Miscellaneous conditions
Idiopathic
Behçet disease Clinical manifestations
•• Oral ulcerationsOral ulcerations (95(95--100%)100%)•• UroUro--genital lesionsgenital lesions (75%)(75%)•• Cutaneous lesionsCutaneous lesions (>75%)(>75%)•• Ocular diseaseOcular disease (25(25--75%)75%)•• Neurologic diseaseNeurologic disease ( < 20%)( < 20%)
•• Oral ulcerationsOral ulcerations (95(95--100%)100%)•• UroUro--genital lesionsgenital lesions (75%)(75%)•• Cutaneous lesionsCutaneous lesions (>75%)(>75%)•• Ocular diseaseOcular disease (25(25--75%)75%)•• Neurologic diseaseNeurologic disease ( < 20%)( < 20%)
20° Congresso Regionale ACOI , 31 ottobre 2008
•• Neurologic diseaseNeurologic disease ( < 20%)( < 20%)•• large Vessel large Vessel Vascular diseaseVascular disease (33%)(33%)•• ArthritisArthritis (75%)(75%)•• Renal diseaseRenal disease (uncommon)(uncommon)•• Cardiac diseaseCardiac disease (uncommon)(uncommon)•• Pulmonary diseasePulmonary disease (uncommon)(uncommon)•• Gastrointestinal SymptomsGastrointestinal Symptoms (common)(common)•• Gastrointestinal DiseaseGastrointestinal Disease (more or less frequent depending on the geographical (more or less frequent depending on the geographical
area: infrequent in in Italy and middle east area: infrequent in in Italy and middle east –– common in far east)common in far east)
•• Neurologic diseaseNeurologic disease ( < 20%)( < 20%)•• large Vessel large Vessel Vascular diseaseVascular disease (33%)(33%)•• ArthritisArthritis (75%)(75%)•• Renal diseaseRenal disease (uncommon)(uncommon)•• Cardiac diseaseCardiac disease (uncommon)(uncommon)•• Pulmonary diseasePulmonary disease (uncommon)(uncommon)•• Gastrointestinal SymptomsGastrointestinal Symptoms (common)(common)•• Gastrointestinal DiseaseGastrointestinal Disease (more or less frequent depending on the geographical (more or less frequent depending on the geographical
area: infrequent in in Italy and middle east area: infrequent in in Italy and middle east –– common in far east)common in far east)