Objective To evaluate the clinical relationship between serum carcinoembryonic antigen (CEA) and mortality of anti-melanoma differentiation associated gene 5 (MDA5) antibody positive dermatomyositis with interstitial lung disease (ILD). MethodsThe consecutive clinical data of 214 patients with anti MDA5 antibody positive dermatomyositis from West China Hospital of Sichuan University from February 2017 to September 2019 were collected retrospectively, including demographic, laboratory examination and imaging examination data. Patients were divided into CEA elevated group (CEA≥4.63 ng/mL) and CEA normal group (CEA<4.63 ng/mL) according to CEA level. R4.1.2 software was used for statistical analysis of all data, and Kaplan Meier method was used to draw the survival curve. Cox proportional hazard model was used to analyze the survival of patients with ILD, and to explore the risk factors associated with the survival of patients with anti-MDA5 antibody positive dermatomyositis with ILD. Results There were 180 patients with ILD who met the inclusion and exclusion criteria, 57 patients with rapidly progressive pulmonary interstitial fibrosis (RPILD), and 123 patients without RPILD; 121 women and 59 men, with an average age of 50.2±10.7 years; The average follow-up was 23.5 months, and 52 patients died. Univariable analysis suggested that CEA≥4.63 ng/mL, smoking, RPILD, lactate dehydrogenase (LDH) ≥321 IU/L, albumin<30 g/L and dyspnea were risk factors associated with death in patients with anti MDA5 dermatomyositis combined with ILD. Multivariable Cox regression analysis showed that CEA≥4.63 ng/mL [hazard ratio (HR) =3.01, 95% confidence interval (CI) 1.23 - 7.32, P=0.015], RPILD (HR=3.87, 95%CI 2.09 - 7.19, P<0.001), smoking (HR=2.37, 95%CI 1.25 - 4.47, P=0.008), LDH≥321 IU/L (HR=2.47, 95%CI 1.23 - 4.96, P=0.011), albumin<30 g/L (HR=2.57, 95%CI 1.38 - 4.78, P=0.003) were independent predictors for mortality. ConclusionsSerum CEA level can be used as a clinical prognostic predictor in patients with anti-MDA5 positive dermatomyositis and ILD. RPILD, smoking, LDH≥321 IU/L, and albumin<30 g/L are independent predictors for mortality.
【摘要】 目的 探讨癫痫持续状态引起的脑部异常MRI表现的特点。 方法 回顾分析2004年—2007年5例患者由癫痫持续状态引起的脑部异常MRI表现，患者均在发作后72 h内行头颅MRI检查，常规进行了T2WI、T1WI、液体衰减反转恢复序列、弥散加权成像（diffusion-weighted imaging，DWI），同时进行了磁共振血管造影（magnetic resonance angiography，MRA）及增强扫描。 结果 5例患者均由部分性发作泛发为全面性强直-阵挛发作，均在发作后行MRI时出现明显单侧大脑半球异常表现，且与部分性发作侧相对应，在T2WI上均表现为局部皮层肿胀，呈高信号，其中3例DWI上出现皮层高信号，病灶不按脑血管分布。4例在MRA上出现病灶同侧大脑中动脉血管较对侧明显增多。5例行增强扫描后均见病灶侧脑膜明显强化。5例患者临床发作后均遗留有与MRI异常病灶相应的临床神经功能缺失。其中3例病情稳定后复查MRI，发现异常病灶及MRA完全恢复正常。 结论 癫痫持续状态引起脑部异常MRI表现特点：①常局限于大脑皮层的T2WI、DWI高信号，且病灶不按血管分布区分布；②MRA提示单侧脑血管增多；③增强扫描见单侧脑膜强化。大部分病灶可逆。这些特点可用来区别是癫痫引起的异常病灶还是脑部的致痫灶。【Abstract】 Objective To summarize the characteristics of abnormal MRI findings of brain caused by status epilepticus. Methods We retrospectively analyzed the abnormal MRI findings of brain in five patients with status epilepticus from 2004 to 2007. All of them received MRI examination within 72 hours after seizures, including the routine examination of T2WI, T1WI, FLAIR and diffusion-weighted imaging (DWI). Meanwhile, MRA and enhancement scanning were carried out. Three of them were reexamined with MRI after the disease came into a stable condition. Results All the five patients transferred from partial seizures initially to generalized tonic clonic seizures, and obvious abnormal MRI findings were detected at the unilateral cerebral hemisphere, corresponding to the side of partial seizures. They appeared regional swelling of cortex with high signal in T2WI, and three cases were found with cortex high signals in DWI, which were not distributed according to cerebral vessels. In four cases, we found that the branches of the middle cerebral artery increased markedly than the contralateral branches on the ipsilateral lesions in MRA. Enhancement scanning also showed that the meninges at the side of the lesion was strengthened significantly in five cases. The loss of neurological functions corresponding to abnormal lesions in MR remained after seizures in five cases among whom three patients recovered completely in terms of their abnormal focuses and MRA findings after their diseases came into a stable condition. Conclusions The features of abnormal brain MRI findings caused by status epilepticus are: ① High signals in T2WI and DWI are confined to the cortex, and the focuses are not distributed according to cerebral vessels; ② MRA shows that unilateral cerebral vessels increases in number; ③ Strengthened unilateral meninges can be observed in enhanced scanning and most of lesions are reversible. According the characteristics, we can distinguish the abnormal lesions caused by epilepsy from focuses in the brain causing epilepsy.