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Gastrointestinal dysmotility complicating Behçet's syndrome: description of a newly recognised clinical phenotype
Desmond P Kidd1, Helen Kibrom2, Elizabeth Ssendi3
1The National Behçet's Syndrome Centre of Excellence, Royal London Hospital, Whitechapel, London, UK. desmond.kidd@nhs.net.
Objectives:
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).
Methods:
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.
Results:
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.
Conclusions:
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.
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