Angioedema associated with Crohnʼs disease: Response to biologics

Abstract

A 46-year-old female patient with terminal ileum Crohn's disease and ankylosing spondylitis presented with recurrent angioedema and urticaria. Investigations ruled out hereditary angioedema, and environmental or food allergen triggers. She was diagnosed with chronic idiopathic urticaria with angioedema, and was treated with a trial of intravenous immunoglobulin immunotherapy, danazol, prednisone and hydroxyzine. Due to ongoing bowel and arthritic complaints, she was started on infliximab infusions and within 2 treatments, she had complete resolution of the angioedema and urticaria, as well as of the bowel and arthritic symptoms. Unfortunately she developed allergic reactions to the infliximab and was switched to another anti-tumor necrosis factor (TNF)-a agent, adalimumab. Since then, she has had no further angioedema or urticaria, and her Crohn's disease has been quiescent. This is the first known case report of chronic idiopathic urticaria with angioedema coexistent with Crohn's disease that was successfully treated with anti-TNF-a agents.

Case Report: Refractory Chronic Spontaneous Urticaria Treated With Omalizumab in an Adolescent With Crohn’s Disease

“…One common thread in the pathophysiology of both diseases is the imbalance in cytokine levels, in particular for IL-17 and TNF-α ( 21 – 23 ). Habal et al ( 24 ) described the first case of CSU with angioedema coexistent with CD that was successfully treated with anti-TNF-α agents. Whether the CSU was cured due to the remission of the CD or because the TNF- α was a common cytokine in the pathogenetic pathway of the two diseases is difficult to understand.…”

“…Whether the CSU was cured due to the remission of the CD or because the TNF- α was a common cytokine in the pathogenetic pathway of the two diseases is difficult to understand. The authors hypothesized that given the similarity of cytokine derangements in CSU and CD, therapies that target the TNF- α could be effective in both conditions ( 24 ). In support of this hypothesis, cases of patients with refractory CSU, who were successfully treated with TNF-α inhibitors, are described in the literature ( 25 , 26 ).…”