Acute Abdominal Pain as the Initial Presentation of an Acquired C1 Inhibitor Deficiency.

GE Port J Gastroenterol

Allergy and Clinical Immunology Department, Centro Hospitalar Universitário do Porto, Porto, Portugal.

Published: August 2024

AI Article Synopsis

  • - Acquired angioedema (AAE) is a rare condition that can cause severe abdominal pain and is often misdiagnosed in emergency rooms, potentially leading to unnecessary treatments.
  • - A case study of a 47-year-old man revealed that initial suspicion of ACEI-induced angioedema was incorrect; instead, tests showed AAE with abnormal C1 inhibitor function and low complement levels.
  • - Recognizing AAE early in patients with acute abdominal symptoms is crucial, as it can indicate underlying serious conditions and lead to better treatment outcomes.

Article Abstract

Introduction: Acquired angioedema (AAE), a rare cause of adult-onset non-urticarial mucocutaneous angioedema, can present as acute abdomen, a frequent complaint in the emergency room (ER), often leading to unnecessary and potentially harmful procedures.

Case Presentation: We report a 47-year-old hypertense male, controlled with an angiotensin converting enzyme inhibitor (ACEI), who presented in the ER with progressively worsening abdominal pain, nausea, and vomiting, and a radiologic workup revealing small intestine thickening, initially diagnosed with ACEI-induced angioedema. However, further investigation revealed low serum levels of C4, C1q, and C1 inhibitors, with an abnormal function of the latter, favoring the diagnosis of AAE instead. The frequent association of this condition with lymphoproliferative disorders encouraged further studies, which unveiled a monoclonal gammopathy IgM/Kappa, representing an increased risk of Waldenström macroglobulinemia, non-Hodgkin lymphoma, and multiple myeloma.

Discussion: AAE should be regarded as an important differential diagnosis in patients presenting with acute abdomen in the ER, especially when more common causes are excluded. A correct and early diagnosis may represent a chance for a better prognosis of underlying diseases.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11305688PMC
http://dx.doi.org/10.1159/000533179DOI Listing

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