Copyright
©The Author(s) 2015.
World J Gastroenterol. Apr 7, 2015; 21(13): 3801-3812
Published online Apr 7, 2015. doi: 10.3748/wjg.v21.i13.3801
Published online Apr 7, 2015. doi: 10.3748/wjg.v21.i13.3801
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 |
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 |
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 |
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] |
- Citation: Skef W, Hamilton MJ, Arayssi T. Gastrointestinal Behçet's disease: A review. World J Gastroenterol 2015; 21(13): 3801-3812
- URL: https://www.wjgnet.com/1007-9327/full/v21/i13/3801.htm
- DOI: https://dx.doi.org/10.3748/wjg.v21.i13.3801