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.
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.
Authors: Alaa N Alsharief; Ronald M Laxer; Qiuyan Wang; Jennifer Stimec; Carina Man; Paul Babyn; Andrea S Doria Journal: Insights Imaging Date: 2020-07-31