Hereditary Angioedema-Associated Acute Pancreatitis in C1-Inhibitor Deficient and Normal C1-Inhibitor Patients: Case Reports and Literature Review

Author(s)

Veronez, Camila Lopes ^[1] ; Campos, Regis Albuquerque ^[2] ; Constantino-Silva, Rosemeire Navickas ^[3] ; Nicolicht, Priscila ^[1] ; Pesquero, Joao Bosco ^[1] ; Grumach, Anete Sevciovic ^[3]

Affiliation

^[1] Univ Fed Sao Paulo, Dept Biophys, Sao Paulo - Brazil

^[2] Univ Fed Bahia, Dept Clin Immunol, Salvador, BA - Brazil

^[3] Fac Med ABC, Dept Clin Immunol, Santo Andre - Brazil

• Document type: Review article
• Source: FRONTIERS IN MEDICINE; v. 6, APR 17 2019.
• Web of Science Citations: 0

Abstract

Abdominal pain due to intestinal swellings is one of the most common manifestations in hereditary angioedema (HAE). Bowel swellings can cause severe abdominal pain, nausea, vomiting, and diarrhea, which may lead to misdiagnosis of gastrointestinal disorders. In rare cases, HAE abdominal attacks can be accompanied by acute pancreatitis. Here, we report 3 patients with HAE and acute pancreatitis and present a literature review of similar cases. Patients with confirmed diagnosis of HAE secondary to C1-inhibitor (C1-INH) deficiency (n = 2) and HAE with normal C1-INH and F12 mutation (F12-HAE) (n = 1) were included. Pancreatitis was diagnosed based on clinical symptoms and high lipase and amylase levels. Three HAE patients were diagnosed with acute pancreatitis based on increased amylase levels during severe abdominal swelling episodes. Two were previously diagnosed with HAE type I and one with F12-HAE. Pancreatitis was efficiently treated in two patients using Icatibant, with pain relief within hours. When conservatively treated, pancreatitis pain took longer time to resolve. Eighteen pancreatitis cases in HAE with C1-INH deficiency were previously reported and none in F12-HAE. Most patients (12/18) underwent invasive procedures and/or diagnostic methods. Although rare, severe abdominal HAE attacks could cause pancreatitis; HAE-specific treatments may be efficient for HAE-associated pancreatitis. HAE should be considered as a differential diagnosis of acute idiopathic pancreatitis. To our knowledge, this is the first report of HAE-associated pancreatitis in a F12-HAE patient treated with Icatibant. (AU)

• FAPESP's process: 15/25494-1 - Development of a gene therapy model for hereditary angioedema based on SERPING1 gene edition by CRISPR-Cas9 system
• Grantee: Camila Lopes Veronez
• Support Opportunities: Scholarships in Brazil - Post-Doctoral
• FAPESP's process: 13/02661-4 - Genetics of hereditary angioedema
• Grantee: João Bosco Pesquero
• Support Opportunities: Regular Research Grants
• FAPESP's process: 14/27198-8 - Establishment of a center of genetic and molecular research for clinical challenges
• Grantee: João Bosco Pesquero
• Support Opportunities: Research Projects - Thematic Grants