Frequency of abdominal surgery in patients with familial Mediterranean fever

Timuçin Kaşifoğlu1, Döndü Usküdar Cansu, Cengiz Korkmaz

  • 1Division of Rheumatology, Deparment of Internal Medicine, Eskisehir Osmangazi University Medical Faculty, Eskişehir, Turkey. tkas@mynet.com

Abstract

Insights

Familial Mediterranean fever (FMF) patients frequently undergo unnecessary abdominal surgeries, often appendectomies, before diagnosis. This highlights the need for careful clinical evaluation to avoid misdiagnosis of appendicitis in FMF patients.

Area of Science:

  • Rheumatology
  • Gastroenterology
  • Surgical Gastroenterology

Background:

  • Familial Mediterranean fever (FMF) is a genetic autoinflammatory disorder characterized by recurrent episodes of serositis, primarily peritonitis.
  • Abdominal manifestations of FMF can mimic acute abdominal emergencies, leading to diagnostic challenges and potential surgical interventions.
  • Misdiagnosis of FMF as acute appendicitis is a common issue, resulting in delayed definitive diagnosis and treatment.

Purpose of the Study:

  • To determine the frequency of abdominal surgeries in patients diagnosed with Familial Mediterranean fever (FMF).
  • To identify the primary reasons for abdominal surgeries performed on FMF patients, particularly before diagnosis.
  • To emphasize the diagnostic challenges posed by FMF and the frequent misdiagnosis of appendicitis.

Main Methods:

  • Retrospective review of medical records for 254 FMF patients (127 males, 127 females).
  • Inclusion of 182 healthy controls (89 males, 93 females) for comparative analysis of abdominal operation rates.
  • Comparison of abdominal surgery frequency, including appendectomy, between FMF patients and healthy controls.

Main Results:

  • A significant proportion of FMF patients (29.1%) underwent abdominal surgery, with 74 patients having 92 operations in total.
  • Appendectomy was the most common initial surgery before FMF diagnosis, performed in 68 FMF patients (26.6%).
  • Abdominal operations and appendectomies were significantly more frequent in the FMF group compared to healthy controls (8.7% and 4.9% respectively, p=0.0001).

Conclusions:

  • Recurrent abdominal attacks in FMF patients can lead to unnecessary laparotomies and misdiagnosis, particularly of appendicitis.
  • FMF patients may present with acute abdominal symptoms even while undergoing treatment with colchicine, necessitating careful clinical assessment.
  • Accurate and timely diagnosis of FMF is crucial to prevent misdiagnosis and subsequent surgical interventions for conditions like appendicitis.

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