Characteristics, Treatment, and Long-Term Outcome of Gastrointestinal Involvement in Behcet's Syndrome

Abstract

Gastrointestinal involvement is rare in Behçet's syndrome (BS) patients from the Mediterranean basin. We report the demographic and disease characteristics, treatment modalities, and outcome of patients with gastrointestinal involvement in BS (GIBS).We retrospectively reviewed the charts of all BS patients in our BS clinic with a diagnosis of GIBS. Patients were invited to the clinic to assess their outcome.Among 8763 BS patients, we identified 60 with GIBS (M/F: 32/28, mean age at diagnosis: 34 ± 10, mean follow-up: 7.5 ± 4 years), after excluding 22 patients with mimicking symptoms. Six (10%) had juvenile-onset BS. The most common intestinal localization was ileocecal region (36/59, 61%) mainly as big oval ulcer/s. Initial treatment was azathioprine for moderate to severe (n = 37) and 5-ASA for mild cases (n = 16). Anti-TNFs and/or thalidomide provided remission in 12 of 18 (67%) refractory patients. Emergency surgery was required in 22 patients. Nine patients did not receive postoperative immunomodulators and 8 relapsed. Overall, 48 of 60 (80%) patients were in remission (29/48 without treatment) at the time of survey. Three recently treated and 2 refractory patients were still active, 3 had died due to non-GI-related reasons, and 4 were lost to follow-up.Careful evaluation for excluding mimickers is important during the diagnosis of GIBS. Azathioprine seems to be a good choice as first-line treatment with high remission rates and few adverse events. Thalidomide and/or TNF-alpha antagonists may be preferred in resistant cases. Surgery may be required for perforations or massive bleeding, and postoperative immunosuppressive treatment is necessary for preventing postoperative recurrences.

2018 update of the EULAR recommendations for the management of Behçet’s syndrome

“…One of the most challenging issues regarding gastrointestinal involvement is to diagnose it correctly since abdominal pain, diarrhoea and intestinal ulcers may commonly be related to other reasons such as non-steroidal anti-inflammatory drug ulcers and gastrointestinal infections including tuberculosis, especially among patients receiving immunosuppressives 91. Confirming the diagnosis is essential to prevent the unnecessary use of immunosuppressives that may be especially harmful if the patient has an infection.…”

“…The evidence available for the management of gastrointestinal involvement relies on retrospective observational data since there are no controlled trials for this relatively uncommon type of involvement 91. The choice of the initial treatment modality depends on the severity of gastrointestinal involvement.…”

“…A cohort study of BS patients with gastrointestinal involvement showed that almost a third of these patients required emergency surgery due to perforation, major bleeding or obstruction 91. Timely recognition of these complications is very important since they may be fatal if left untreated.…”

Behçet-Syndrom

“…Als mittlere Manifestation werden die anteriore Uveitis, die milde posteriore Uveitis, die milde Großgefäßvaskulitis (Venenthrombosen, Thrombophlebitiden, Arteriitis kleiner und mittlerer Gefäße), die milde gastrointestinale Beteiligung und die chronischrezidivierende Arthritis angesehen. Primär empfohlen werden hier die klassischen Immunsuppressiva wie Azathioprin, Ciclosporin A und Methotrexat mit und ohne systemische Glukokortikoide [56,80,[84][85][86][87] und zumindest zeitweise topisch wirksame Glukokortikoide bei der anterioren Uveitis.…”

Behçet-Syndrom

“…Primär empfohlen werden hier die klassischen Immunsuppressiva wie Azathioprin, Ciclosporin A und Methotrexat mit und ohne systemische Glucokortikoide. [56,80,[84][85][86][87] und zumindest zeitweise topisch wirksame Glucokortikoide bei der anterioren Uveitis.…”