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Background  Adamantiades-Behçet’s Disease (ABD) is an immunological recurrent systemic vasculitis with a chronic course. We investigated the predictors of long-term flare-ups, poor outcomes and event-free survival in Chinese non-surgical patients with intestinal ABD.    Methods  This was a prospective cohort study of 109 intestinal ABD patients seen in our institution between October 2012 and January 2019 who met the international criteria for ABD and had intestinal ulcers confirmed on colonoscopy. Predictors of relapses and poor outcomes, event-free survival were calculated using logistic regression models and Cox proportional hazard regression models, respectively.    Results  Sixty-six intestinal ABD patients (60.55%) had ileocecal ulcers; 19 patients (17.43%) presented with colorectum ulcers; 24 patients (22.02%) showed both ileocecal and colorectum ulcers. 7 patients (6.42%) experienced at least 1 flare-up of intestinal ulcers. 38 patients (34.86%) complained of non-healing intestinal ulcers. In multivariate analysis, location of intestinal ulcers (ileocecal and colorectum) (odd ratio (OR) 7.498 [95% confidence interval [95% CI] 1.844–30.480]), erythrocyte sedimentation rate (ESR) > 24 mm/h (OR 5.966 [95% CI 1.734–20.528]), treatment with infliximab (IFX) (OR 0.130 [95% CI 0.024–0.715]), and poor compliance (OR 11.730 [95% CI 2.341–58.781]) were independently correlated with a poor outcome. After a median follow-up of 28 months, 45 intestinal ABD patients (41.28%) underwent adverse events. Factors independently associated with shorter event-free survival were early onset of ABD (< 7 years) (hazard ratio (HR) 2.431 [95% CI 1.240–4.764]) and poor compliance (HR 3.058 [95% CI 1.612–5.800]).    Conclusion  Distribution of intestinal ulcers (ileocecal and colorectum), ESR > 24 mm/h, treatment without IFX, and poor compliance were independent risk factors for poor outcomes in non-surgical intestinal ABD patients.

orphanet journal of rare diseases

Poor prognostic factors in patients with newly diagnosed intestinal Adamantiades-Behçet’s disease in the Shanghai Adamantiades-Behçet’s disease database: a prospective cohort study