Ornek Ahmet, Kurucay Mustafa, Henning Bernhard F, Pagonas Nikolaos, Schlottmann Renate, Schmidt Wolfgang E, Giese Arnd
Department of Internal Medicine I, Marienhospital Herne, Medical Center of Ruhr University Bochum, Herne, Germany (A.Ö., M.K., B.F.H., N.P., A.G.); Institute for Diagnostic and Interventional Radiology and Nuclear Medicine, University Medical Center Bergmannsheil, Ruhr University Bochum, Bochum, Germany (A.Ö.); and Department of Internal Medicine I, St Josef-Hospital, Medical Center of Ruhr University Bochum, Bochum, Germany (R.S., W.S., A.G.).
J Ultrasound Med. 2014 Nov;33(11):1991-7. doi: 10.7863/ultra.33.11.1991.
Familial Mediterranean fever (FMF) can be associated with splenomegaly. Prospective quantitative data are lacking. We performed a sonographic assessment of spleen size in patients with FMF and healthy control participants to assess its diagnostic value.
Patients with FMF according to the criteria of Livneh et al (Arthritis Rheum 1997; 40:1879-1885) who were in an asymptomatic interval and control participants were prospectively included in this study in Germany and underwent sonographic measurement of the spleen as well as a structured interview and a physical examination. Patients and controls were Turkish migrants.
Thirty-six patients and 27 controls were included. Patients and controls did not differ significantly in age (mean ± SD, 34.8 ± 9.7 versus 33.3 ± 10.0 years, respectively; P = .56), sex, height, weight, or body mass index (26.7 ± 4.7 versus 26.1 ± 4.3 kg/m(2); P = .63). Spleen size was greater in patients than controls in width (4.3 ± 1.0 versus 3.7 ± 0.7 cm; P = .008) and also length (12.1 ± 1.9 versus 10.5 ± 1.4 cm; P = .001). Twenty-six of 36 patients (72.2%) had a history of appendectomy compared to 3 of 27 controls (11.1%; P < .001). The combination of an enlarged spleen (length >11 cm and/or width >4 cm) gave specificity of 100% (95% confidence interval, 87%-100%) and a positive predictive value of 100% (95% confidence interval, 78%-100%) for the diagnosis of FMF in our study.
Spleen size as evaluated by sonography is larger in patients with FMF compared to healthy controls. Most patients with FMF included in this study had undergone appendectomy. Familial Mediterranean fever should be considered as a differential diagnosis in Turkish migrants in Germany if the spleen is enlarged and a history of appendectomy is reported.
家族性地中海热（FMF）可能与脾肿大有关。目前缺乏前瞻性定量数据。我们对FMF患者和健康对照者进行了脾脏大小的超声评估，以评估其诊断价值。
根据Livneh等人（《关节炎与风湿病》1997年；40:1879 - 1885）的标准，处于无症状期的FMF患者和对照者被前瞻性纳入德国的这项研究，并接受脾脏超声测量以及结构化访谈和体格检查。患者和对照者均为土耳其移民。
纳入36例患者和27例对照者。患者和对照者在年龄（平均±标准差，分别为34.8±9.7岁和33.3±10.0岁；P = 0.56）、性别、身高、体重或体重指数（26.7±4.7与26.1±4.3 kg/m²；P = 0.63）方面无显著差异。患者的脾脏宽度（4.3±1.0与3.7±0.7 cm；P = 0.008）和长度（12.1±1.9与10.5±1.4 cm；P = 0.001）均大于对照者。36例患者中有26例（72.2%）有阑尾切除术史，而27例对照者中有3例（11.1%）有阑尾切除术史（P < 0.001）。在我们的研究中，脾脏肿大（长度>11 cm和/或宽度>4 cm）的联合指标对FMF诊断的特异性为100%（95%置信区间，87% - 100%），阳性预测值为100%（95%置信区间，78% - 100%）。
与健康对照者相比，FMF患者经超声评估的脾脏大小更大。本研究纳入的大多数FMF患者都接受过阑尾切除术。在德国的土耳其移民中，如果脾脏肿大且有阑尾切除术史，应考虑将家族性地中海热作为鉴别诊断。
