Gastrointestinal dysmotility complicating Behçet's syndrome: description of a newly recognised clinical phenotype.

Abstract

Gastrointestinal dysmotility may arise in a variety of auto-immune and auto-inflammatory diseases and hitherto has not been described in Behçet's syndrome (BS). We present data on a cohort of seven patients under our care who presented with symptoms of and investigations compatible with an immune associated disorder of gastrointestinal motility, or enteric neuropathy. We describe the clinical features and investigation results. We undertook a trial of a novel treatment in the disease, apheresis, and noted a response not only to the enteric neuropathy but also to the systemic features of the disease, despite previous maximal immunosuppressive therapy in most cases. Gastrointestinal dysmotility may arise in BS and is effectively treated by apheresis. The mechanism by which this response is made immunologically requires to be elucidated in future studies.