Imaging findings of familial Mediterranean fever
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Published on: June 20, 2014
Gisele E Ishak1, Nabil J Khoury, Ghina A Birjawi
1Department of Diagnostic Radiology, American University of Beirut Medical Center, P.O. Box 11-0236, Beirut, Lebanon.
Purpose:
The aim of this study was to study the imaging findings of familial Mediterranean fever (FMF).
Materials And Methods:
We performed a retrospective review of the medical records and imaging studies of 38 patients with proven FMF, diagnosed between 1992 and 2002.
Results:
The most common clinical manifestation was recurrent peritoneal attacks with abdominal pain (76.3%) and fever (42.1%). Abdominal imaging findings included ileus (n=12), splenomegaly (n=5), hepatomegaly (n=2), ascitis (n=2), focal peritonitis (n=2), mesenteric streaking (n=1), and enlarged mesenteric lymph node (n=1). One patient developed fatal peritoneal mesothelioma, and 13.1% of the patients developed amyloidosis with sonographic findings of renal parenchymal disease or cardiomyopathy. Arthritis was second in frequency, occurring in 34.2% of patients; radiographs were normal (n=4) or showed joint effusion and periarticular soft tissue swelling (n=4) due to synovitis. One patient developed seronegative destructive arthropathy. Skin lesions were noted in 23.6% of patients. Pleuritis was encountered in 13.1% and pericarditis in 5.2%. Polyarteritis nodosa (PAN) was present in two patients, multiple sclerosis in one, and autoimmune hemolytic anemia in one patient.
Conclusion:
FMF predominantly involves abdominal viscera but can affect other organs. The majority of patients have nonspecific imaging findings, and the radiologic diagnosis is rarely considered. Amyloidosis, mesothelioma, and destructive arthropathy are potential serious complications of FMF. PAN, multiple sclerosis, and autoimmune hemolytic anemia are probably rare associations or rather than coincident with FMF.
Insights
Familial Mediterranean fever (FMF) primarily affects abdominal organs, causing nonspecific imaging findings. Serious complications like amyloidosis and mesothelioma can occur, impacting diagnosis and patient outcomes.
Area of Science:
- Rheumatology
- Internal Medicine
- Radiology
Background:
- Familial Mediterranean fever (FMF) is a genetic autoinflammatory disorder.
- Clinical manifestations of FMF are diverse, often mimicking other conditions.
- Imaging plays a role in diagnosing FMF complications but findings are often nonspecific.
Purpose of the Study:
- To investigate and characterize the imaging findings associated with familial Mediterranean fever (FMF).
- To correlate imaging findings with clinical manifestations and complications of FMF.
Main Methods:
- Retrospective review of medical records and imaging studies.
- Analysis of data from 38 patients diagnosed with FMF between 1992 and 2002.
Main Results:
- Recurrent peritoneal attacks were the most common presentation (76.3%).
- Abdominal imaging revealed ileus, splenomegaly, hepatomegaly, ascites, and peritonitis.
- Complications included amyloidosis (13.1%) with renal or cardiac changes, and arthritis (34.2%).
Conclusions:
- FMF predominantly involves abdominal viscera but can affect other organs.
- Imaging findings in FMF are often nonspecific, complicating radiologic diagnosis.
- Amyloidosis, mesothelioma, and destructive arthropathy are significant potential complications of FMF.
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