Ishak Gisele E, Khoury Nabil J, Birjawi Ghina A, El-Zein Youssef R, Naffaa Lena N, Haddad Maurice C
Department of Diagnostic Radiology, American University of Beirut Medical Center, P.O. Box 11-0236, Beirut, Lebanon.
Clin Imaging. 2006 May-Jun;30(3):153-9. doi: 10.1016/j.clinimag.2005.07.002.
The aim of this study was to study the imaging findings of familial Mediterranean fever (FMF).
We performed a retrospective review of the medical records and imaging studies of 38 patients with proven FMF, diagnosed between 1992 and 2002.
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.
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.
本研究旨在探讨家族性地中海热（FMF）的影像学表现。
我们对1992年至2002年间确诊的38例经证实的FMF患者的病历和影像学研究进行了回顾性分析。
最常见的临床表现是反复出现的腹膜发作伴腹痛（76.3%）和发热（42.1%）。腹部影像学表现包括肠梗阻（n = 12）、脾肿大（n = 5）、肝肿大（n = 2）、腹水（n = 2）、局灶性腹膜炎（n = 2）、肠系膜条纹（n = 1）和肠系膜淋巴结肿大（n = 1）。1例患者发生致命性腹膜间皮瘤，13.1%的患者发生淀粉样变性，超声表现为肾实质疾病或心肌病。关节炎的发生率次之，为34.2%；X线片正常（n = 4）或显示由于滑膜炎导致的关节积液和关节周围软组织肿胀（n = 4）。1例患者发生血清阴性破坏性关节病。23.6%的患者有皮肤病变。胸膜炎发生率为13.1%，心包炎发生率为5.2%。2例患者有结节性多动脉炎（PAN），1例有多发性硬化，1例有自身免疫性溶血性贫血。
FMF主要累及腹部脏器，但也可影响其他器官。大多数患者有非特异性影像学表现，很少考虑进行放射学诊断。淀粉样变性、间皮瘤和破坏性关节病是FMF潜在的严重并发症。PAN、多发性硬化和自身免疫性溶血性贫血可能是罕见的关联，而非与FMF巧合。
