Kyung-Jo Kim

doi:10.4078/jrd.2014.21.4.176

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Kim: Update on the Treatment of Intestinal Behçet's Disease

Review Article

J Rheum Dis 2014;21(4):176-181.

Published online: 31 October 2014

DOI: https://doi.org/10.4078/jrd.2014.21.4.176

Update on the Treatment of Intestinal Behçet's Disease

Kyung-Jo Kim

Department of Gastroenterology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea

Corresponding to: Kyung-Jo Kim, Department of Gastroenterology, University of Ulsan College of Medicine, Asan Medical Center, 88 Olympic-ro 43-gil, Songpa-gu, Seoul 138-736, Korea. E-mail: capsulendos@gmail.com

Received 22 June 2014 Revised 29 June 2014 Accepted 30 June 2014

This is a Free Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Behçet's disease is a chronic relapsing disease with unknown etiology, involving the multiorgan system characterized clinically by oral and genital aphthae, cutaneous lesions, and ophthalmologic, neurologic, or gastrointestinal manifestations (or some combination of these). Since intestinal Behçet's disease has been treated anecdotally with various therapeutic modalities, clinical evidence regarding the management of intestinal Behçet's disease is still lacking. 5-aminosalycylic acid, corticosteroids, immunosuppressants, and surgical therapy have been considered as traditional therapies for intestinal Behçet's disease. The treatment with thalidomide and anti-TNF agents, such as infliximab or adalimumab, is increasing. Future clinical trials are still needed.

Keywords: Intestine, Behçet's disease

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Figure 1.

Colonoscopic findings of typical intestinal Behcet's disease. Single round to oval shaped ulcer with clean ulcer base is noted in the terminal ileum.

Figure 2.

A suggested algorithm for the treatment of intestinal Behç et's disease. *Refractory abdominal pain and diarrhea on 5-aminosalcylates. ^#5-ASA, 5-amino salicylicacids; PD, prednisolone; AZA/6MP, azathioprine/6-mercap-topurine; anti-TNF agents, anti-tumor necrosis factor.

Table 1.

Guideline statements for diagnosis and management of intestinal Behçet's disease (7)

1. Diagnosis of intestinal Behçet's disease can be made if

A. There is a typical oval-shaped large ulcer in the terminal ileum, OR

B. There are ulcerations or inflammation in the small or large intestine,

AND clinical findings meet the diagnostic criteria of Behçet's disease

2. Tuberculosis, Crohn's disease, nonspecific colitis, drug-asso-ciated colitis and other diseases that mimic intestinal Behçet's disease should be excluded before diagnosis of intestinal Behçet's disease is made

3. Typical clinical findings of Behçet's disease such as abdominal pain, hematochezia, oral aphthae, cutaneous lesion, genital ulcer, and arthralgia should be sought. These findings may not appear at one time

4. Minimally, colonoscopy with biopsy, double-contrast barium enema, and laboratory tests are necessary to diagnose intestinal Behçet's disease. When intestinal Behçet's disease is suspected, EGD and enteroclysis are needed to determine the extent of gastrointestinal lesions

5. When intestinal Behçet's disease is suspected, dermatology, ophthalmology, orthopedics, and neurology consults are necessary

6. There is no clear classification of severity for intestinal Behçet's disease. Severity of disease is determined based on systematic symptoms, degree of abdominal symptoms, depth of intestinal ulcerations, presence of bleeding from intestinal ulcerations, laboratory findings such as WBC, C-reactive protein, and degree of anemia

Table 2.

Conventional and emerging treatment of intestinal Behçet's disease

Medications	Indications	Recommended dose
Conventional treatment
Colchicine	Mucocutaneous symptoms	0.5∼1.5 mg
Sulphasalazine	Mild symptoms	3∼4 g/day
Mesalazine	Mild symptoms	2.25∼3 g/day
Corticosteroids	Severe systemic symptoms, abdominal symptoms, and diarrhea	PD 0.5∼1 mg/Kg/day for 1∼2 weeks
Azathioprine	Steroid-dependent	2∼2.5 mg/Kg/ day
	Steroid-refractory
Emerging treatment
Thalidomide		100∼300 mg/day
Anti TNF-α agent
-Infliximab		5 mg/Kg at week 0, 2, 6, and q 8 weeks
-Adalimumab		160 mg at week 0, 80 at week 2, 40 mg every other week

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