Development of consensus statements for the diagnosis and management of intestinal Behçet's disease using a modified Delphi approach.

BACKGROUND: Although intestinal Behçet's disease has been treated anecdotally with various therapeutic modalities, clinical evidence regarding management of intestinal Behçet's disease is lacking. The objective of this study was to develop consensus-based practice guidelines for diagnosis and treatment of intestinal Behçet's disease by using a modified Delphi approach. METHODS: Three groups of Japanese gastroenterology specialists were involved in the study: moderators, an expert panel, and a professional group. Clinical statements for ratings were extracted from relevant literature, a survey of the professional group, and by discussion among the expert panel. The expert panel rated the clinical statements according to a nine point scale. After the first round of ratings, a panelist meeting was held to discuss areas of disagreement and to clarify areas of uncertainty. The list of clinical statements was revised after the panelist meeting, and a second round of rating was conducted. RESULTS: Thirty-two relevant articles were selected in a literature search, and 35 clinical statements were extracted. An additional 209 clinical statements were developed from the survey and discussion among gastroenterology specialists. In the first and second rounds, 56% and 60% of statements, respectively, received median scores > or =7. The range of scores decreased considerably from the first to the second round. CONCLUSIONS: 5-Aminosalycylic acid, corticosteroids, immunosuppressants, enteral nutrition, total parenteral nutrition, and surgical therapy were considered standard therapy for intestinal Behçet's disease. Infliximab, colchicines, thalidomide, other pharmacological therapy, endoscopic therapy, and leukocytapheresis were deemed experimental therapy. Based on a two-round modified Delphi approach, practice guidelines for diagnosis and treatment of intestinal Behçet's disease were developed.

[A case of Turner syndrome complicated with Crohn's disease, erythema nodosum and Hashimoto disease].

A 26-year-old woman, who had had Turner syndrome from age 10 years old, had diarrhea, fever, joint pain, and erythema in the lower left leg. She was given a diagnosis of Crohn's disease, erythema nodosum, and Hashimoto disease. Systemic steroid therapy was very effective for both intestinal and skin lesions. It has been reported that half of inflammatory bowel disease patients with Turner syndrome have 46XiX (q) type chromosome abnormality, and this case also has this type of abnormality.

Ulcerative colitis followed by the development of Behçet's disease.

A 34-year-old man who had a history of ulcerative colitis (UC) was admitted to our hospital with complaints of arthralgia, erythema nodosum, recurrent oral aphthous ulcers and bloody stools. A colonoscopy revealed multiple aphthous ulcers on his cecum and colon and also revealed a transmural ulcer on his rectum consistent with a diagnosis of UC. The patient was HLA-B51 positive. Based on clinical evidence [recurrent oral ulcers, skin lesions (erythema nodosum), positivity for pathergy test] this patient was diagnosed as having Behçet's disease with gastrointestinal involvement. We describe this rare case of Behçet's disease with colitis and discuss the difficulties in making a differential diagnosis between Behçet's disease and the inflammatory bowel diseases.

Cytokine profile in colonic mucosa of ulcerative colitis correlates with disease activity and response to granulocytapheresis.

OBJECTIVE: The aim of this study was to clarify the correlation between cytokine profile in colonic mucosa with disease activity and response to granulocytapheresis (GCAP) in patients with ulcerative colitis (UC), using a reliable, reproducible quantitative method. METHODS: Colonoscopic biopsies of inflamed colonic mucosa (16 patients, 21 cases) and uninflamed colonic mucosa (25 patients, 33 cases) were obtained from UC patients. Messenger (m)RNA was extracted and subjected to realtime polymerase chain reaction for quantitative measurement of interleukin (IL)-12, interferon-gamma, tumor necrosis factor-alpha, IL-4, IL-8, and IL-18 mRNAs. In seven patients with high disease activity despite prednisolone (PSL) treatment (> or = 20 mg/day), one course of GCAP was conducted, and pre- and post-GCAP cytokine profiles were determined. RESULTS: In inflamed colonic mucosa of UC patients, three cytokine profiles were observed: 1) high expression of interferon-gamma, tumor necrosis factor-alpha, and IL-4 mRNAs but low expression of IL-8 mRNA; 2) high expression of IL-8 mRNA and low expression of others; and 3) low expression of all cytokines examined. Inflamed colonic mucosa of patients with high disease activity showed the second pattern. Inflamed colonic mucosa of patients who were not treated with PSL and who had low disease activity showed the first pattern, whereas those on high-dose PSL exhibited the second pattern. IL-8 mRNA was significantly higher in inflamed UC samples than in uninflamed samples. GCAP was effective in five of seven PSL-resistant patients (71.4%). IL-8 was the only cytokine that correlated with effectiveness of GCAP. Compared with GCAP nonresponders, responders had significantly higher IL-8 mRNA before GCAP and showed marked reduction of IL-8 mRNA after GCAP. CONCLUSIONS: IL-8 mRNA was significantly increased in inflamed mucosa of UC. Patients with high IL-8 mRNA expression in colonic mucosa despite PSL treatment were responsive to GCAP. Therefore, quantitative measurement of mucosal IL-8 mRNA may be useful in predicting the response to GCAP.