Adhesive small-bowel obstruction as a challenging complication of familial Mediterranean fever: A case-based review

Int J Rheum Dis. 2024 Jan;27(1):e14867. doi: 10.1111/1756-185X.14867. Epub 2023 Aug 14.

Abstract

Familial Mediterranean fever (FMF) is the most common inherited autoinflammatory disorder, characterized by recurrent and self-limiting episodes of fever and serosal inflammation. Recurrent serositis may rarely lead to the formation of adhesions in the peritoneum, which may result in mechanical bowel obstruction. The symptoms, such as abdominal pain and vomiting, may mimic typical FMF attacks, resulting in misdiagnosis and severe morbidity, including strangulation and intestinal necrosis. Physicians are generally aware of other complications associated with FMF but reports on peritoneal adhesions and intestinal obstruction in English-language literature are inadequate to increase clinicians' awareness. Therefore, it is crucial to meticulously evaluate FMF patients presenting with abdominal pain and ileus because these symptoms could be due to adhesive small-bowel obstruction (ASBO). Furthermore, patients presenting with ASBO without a history of abdominal surgery should also be thoroughly evaluated, especially as it could be an initial presentation for an autoinflammatory disease. Herein, we present a pediatric case of FMF with the M694V homozygous mutation, complicated by ASBO while under colchicine treatment. Additionally, we provide a comprehensive review of the available literature on ASBO in FMF.

Keywords: adhesive small-bowel obstruction; familial Mediterranean fever; peritoneal adhesions.

Publication types

  • Review
  • Case Reports

MeSH terms

  • Abdominal Pain / etiology
  • Child
  • Colchicine
  • Familial Mediterranean Fever* / complications
  • Familial Mediterranean Fever* / diagnosis
  • Familial Mediterranean Fever* / drug therapy
  • Homozygote
  • Humans
  • Intestinal Obstruction* / diagnosis
  • Intestinal Obstruction* / etiology
  • Intestinal Obstruction* / surgery

Substances

  • Colchicine