Aortic Flow Propagation Velocity in Patients with Familial Mediterranean Fever: an Observational Study

BACKGROUND AND OBJECTIVES: Systemic inflammation has an important role in the initiation of atherosclerosis, which is associated with arterial stiffness (AS). Aortic flow propagation velocity (APV) is a new echocardiographic parameter of aortic stiffness. The relationship between systemic inflammation and AS has not yet been described in patients with familial Mediterranean fever (FMF). We aimed to investigate the early markers of AS in patients with FMF by measuring APV and carotid intima-media thickness (CIMT). SUBJECTS AND METHODS: Sixty-one FMF patients (43 women; mean age 27.3±6.7 years) in an attack-free period and 57 healthy individuals (36 women; mean age 28.8±7.1 years) were included in this study. The individuals with atherosclerotic risk factors were excluded from the study. The flow propagation velocity of the descending aorta and CIMT were measured to assess AS. RESULTS: APV was significantly lower (60.2±16.5 vs. 89.5±11.6 cm/sec, p<0.001) and CIMT was significantly higher (0.49±0.09 vs. 0.40±0.10 mm, p<0.001) in the FMF group compared to the control group. There were significant correlations between APV and mean CIMT (r=-0.424, p<0.001), erythrocyte sedimentation rate (ESR) (r=-0.198, p=0.032), and left ventricle ejection fraction (r=0.201, p=0.029). APV and the ESR were independent predictors of FMF in logistic regression analysis (OR=-0.900, 95% CI=0.865-0.936, p<0.001 and OR=-1.078, 95% CI=1.024-1.135, p=0.004, respectively). Mean CIMT and LVEF were independent factors associated with APV in linear regression analysis (β=-0.423, p<0.001 and β=0.199, p=0.017, respectively). CONCLUSION: We demonstrated that APV was lower in FMF patients and is related to CIMT. According to our results, APV may be an independent predictor of FMF.

A Comparison of Ultrasonography, Computerised Tomography, and Conventional MRI Findings for Splenic Nodules Associated with Type 1 Gaucher s Disease with Diffusion-Weighted MRI Findings

A 26-year-old female patient with Type 1 Gaucher’s disease (GD) was admitted to our clinic with complaints of stomachache and signs of anemia. The patient underwent ultrasonography (US), computerised tomography (CT), and magnetic resonance imaging (MRI) scan. Imaging studies revealed massive hepatosplenomegaly, choledocolithiasis, and six nodules in the spleen with a mean size of 14 mm. The nodules appeared hyperechoic, hypoechoic, and of mixed echogenicity on the US and hypodense on the CT. While the nodules were observed to be iso-hypointense in T1-weighted (T1WI) images, they appeared to be hyperintense in the T2- weighted (T2WI) images. There were no diffusion restrictions in these nodules that appeared on the diffusion-weighted magnetic resonance imaging (DWI). A nodule located at the lower pole was observed to be hypointense in the T2WI images. The nodule located at the lower pole, which appeared hypointense in T2WI series, had restricted diffusion upon DWI. In this study, we aimed to present the properties of splenic GD nodules using US, CT, and conventional MRI, together with DWI. This case report is the first to apply US, CT, and conventional MRI, together with DWI, to the splenic nodules associated with Gaucher’s disease.