Gastrointestinal Behçet's disease: A review

Table 1 Common clinical manifestations of Behçet’s disease[12,130]

Skin, Mucocutaneous	Papulopustular lesions (Behçet’s pustulosis), erythema nodosum, superficial thrombophlebitis, minor aphthous ulcers
Eyes	Anterior and posterior uveitis, retinal vasculitis
Vascular	Deep venous thrombosis, large-vein thrombosis, pulmonary artery aneurysm
Musculoskeletal	Arthralgia, arthritis (monoarticular, oligoarticular)
Gastrointestinal	Ileocecal ulcers
Genitourinary	Genital ulcers, epididymitis
Central nervous system	Meningoencephalitis, parenchymal disease (pyramidal signs, hemiparesis, behavioral changes, sphincter disturbance), intracranial hypertension secondary to dural sinus thrombosis

Table 2 Differences between intestinal Behçet’s disease and Crohn’s disease[39,40,48,49,52,54]

	Crohn’s disease	Intestinal BD
Extra-intestinal manifestations	Iritis, episcleritis more specific	Oral and genital ulcers more common, papulopustular lesions, neurologic and arterial manifestations
Perianal disease (fistula, fissures)	Common	Rare
Strictures, fistula, abscess	Common, characteristic of disease process	Less common but possible
Serologic markers	Anti-saccharomyces cerevisiae antibody (Prevalence: 41%-76%)	IgM anti-α-enolase antibody (Prevalence: 67.5%)
Endoscopic features	Irregular, longitudinal ulcers with cobblestone appearance, may have aphthous lesions Segmental or diffuse involvement	Round or oval shaped, punched-out lesions with discrete margins, > 1 cm, Focal distribution, < 5 ulcers. No aphthous lesions
Pathognomonic lesions on histopathology	Non-caseating epithelioid granuloma	Non-specific neutrophilic or lymphocytic phlebitis with or without aortitis

BD: Behçet’s disease.

Table 3 Disease activity index for intestinal Behçet’s disease

Item	Score
General well-being in the preceding week
Well	0
Fair	10
Poor	20
Very poor	30
Terrible	40
Fever
< 38 °C	0
≥ 38 °C	10
Extraintestinal manifestations	5 points for each manifestation1
Abdominal pain in the preceding week
None	0
Mild	20
Moderate	40
Severe	80
Abdominal mass
None	0
Palpable mass	10
Abdominal tenderness
None	0
Mildly tender	10
Moderately or severely tender	20
Intestinal complications	10 points for each complication2
Number of liquid stools in the preceding week
0	0
1-7	10
8-21	20
22-35	30
≥ 36	40
Total score
Severity of disease
Quiescent Intestinal BD	≤ 19
Mild intestinal BD	20-39
Moderate intestinal BD	40-74
Severe intestinal BD	≥ 75

¹Five points are added for each type of the following manifestations: oral ulcer, genital ulcers, eye lesions, skin lesions or arthralgia. 15 points are added for each of the following: vascular involvement or central nervous system involvement;

²Fistula, perforation, abscess or intestinal obstruction. Adapted from Cheon et al[89]. BD: Behçet’s disease.

Table 4 Summary of evidence for medical management of intestinal Behçet’s disease

Therapy	Level(s) of published evidence
5-ASA/sulfasalazine	Retrospective cohort study[95]
Corticosteroids	Expert opinion, European League Against Rheumatism Recommendations[114]
Thalidomide	Case reports[131-133]
Azathioprine, 6-MP	Retrospective cohort studies[97,123]
Mycophenolate	Case report[134]
Methotrexate	Case series[135]
Tacrolimus	Case report[136]
Infliximab	Single arm clinical trial[102], Retrospective cohort study[104]
	Case series[101]
Adalimumab	Prospective, non-placebo controlled clinical trial[110]
Etanercept	Case report[112]