Comparison of idiopathic recurrent acute pancreatitis [IRAP] and recurrent acute pancreatitis with genetic mutations

Luisa M. Cruz, Joshua Y. Kwon, Sven P. Oman, Himesh Zaver, Gabriel A. Bolaños, Paul T. Kröner, Massimo Raimondo, Yan Bi, Frank J. Lukens, Juan E. Corral

Research output: Contribution to journalArticlepeer-review


Background: Idiopathic recurrent acute pancreatitis (IRAP) describes frequent episodes of pancreatitis without an etiology found using current testing. We compared the natural history of IRAP with recurrent acute pancreatitis with genetic mutations. Methods: Retrospective cohort of patients with recurrent acute pancreatitis (≥2 episodes) and negative conventional testing. All patients had ≥1 episode after cholecystectomy and completed genetic testing. Primary outcomes were chronic pancreatitis incidence, pancreatic cancer, and mortality. Secondary outcomes included opioid and ERCP utilization. Results: 128 patients met criteria for presumed IRAP. 35 patients met criteria for true IRAP. 12 patients had recurrent acute pancreatitis with gene mutations. Chronic pancreatitis developed in 27 (77.1%) IRAP patients over a median of 6 years. Chronic pancreatitis incidence was similar in IRAP and CFTR mutation carriers; but developed later in SPINK1 carriers. No patients developed pancreatic cancer or died from pancreatic-related causes. Patients were frequently treated with oral opioids and ERCP, without significant differences within or between groups. Conclusion: IRAP and pancreatitis in mutation carriers is associated with chronic pancreatitis. Important differences in natural history were observed, but no association was found with cancer or pancreas-related mortality. Efforts to understand the genetic contributions to IRAP, minimize opioids and unnecessary ERCPs are encouraged.

Original languageEnglish (US)
Pages (from-to)1294-1300
Number of pages7
JournalDigestive and Liver Disease
Issue number10
StatePublished - Oct 2021


  • Acute pancreatitis
  • Chronic pancreatitis
  • ERCP
  • Gene mutations
  • Opioids

ASJC Scopus subject areas

  • Hepatology
  • Gastroenterology


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