Abstract
Abstract Background Abdominal pain is a common gastroenterological symptom with an extensive deferential diagnoses. Angioedema is an important cause to remember after the more common causes have been excluded.It is caused by a deficiency in the inhibitor of the first component of classical complement pathway and is divided into hereditary or acquired.Acquired angioedema is associated with autoimmune or lymphoproliferative neoplasms.The clinical features of both include recurrent,self-limiting and circumscribed edema affecting the subcutaneous tissue of the upper respiratory airways and gastrointestinal tract. Aims We describe an interesting case of acquired angioedema presenting with recurrent abdominal pain. We also systematically review the current literature on this rare entity. Methods A search of electronic databases was performed inclusive to September 2019, for all studies and reviews of patients with acquired angioedema manifested as recurrent attacks of abdominal pain. Results: Case Report: 65 year old lady ho has an at least 2 year long history of abdominal pain and bloating associated on some occasions with nausea and vomiting.She presented to our emergency department in August,2018 with a day history of severe generalized abdominal pain and was found to have circumferential thickening,edema with mucosal hyperenhancement involving the distal segment of the small bowel.Splenomegaly was noted on that study.A double balloon enteroscopy was planned,but ultimately cancelled as her ileitis had resolved.The patient was discharged home as her pain resolved with conservative management which included intravenous fluid, pain medications and antiemetics.She then represented to the hospital on September 16, 2019 severe abdominal pain and throat tightness associated with shortness of breath. Again,noted was an extremely short segment of ileum with mucosal edema and hyperenhancement which resolved on a repeat CT scan done during that admission on September 18th, 2019.In light of the patient’s symptoms,CT scan findings,progressive anemia, thrombocytopenia and splenomegaly. It was thought that her abdominal pain is related to secondary to angioedema driven by a lymphoproliferative process as evident by the splenomegaly and worsening cytopenia. A bone marrow was done which revealed clonal B, so the diagnosis of lymphoma was made. The patient’s C1 esterase was 0.2 with reduced functional activity.CH50 less 10 perecent. A diagnosis of acquired angioedema was made and the patient was started on BERINERT 3000 IU SQ every three days with 1500 IU for break through with significant improvement in the frequency and severity of abdominal pain episodes. Conclusions Acquired angioedema is to be considered as a cause of recurrent abdominal pain in a patient with a lymphoproliferative malignancy after common causes and etiologies have been ruled out. Funding Agencies None
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More From: Journal of the Canadian Association of Gastroenterology
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