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 探討癲癇持續狀態引起的腦部異常MRI表現的特點。 方法 回顧分析2004年—2007年5例患者由癲癇持續狀態引起的腦部異常MRI表現，患者均在發作后72 h內行頭顱MRI檢查，常規進行了T2WI、T1WI、液體衰減反轉恢復序列、彌散加權成像（diffusion-weighted imaging，DWI），同時進行了磁共振血管造影（magnetic resonance angiography，MRA）及增強掃描。 結果 5例患者均由部分性發作泛發為全面性強直-陣攣發作，均在發作后行MRI時出現明顯單側大腦半球異常表現，且與部分性發作側相對應，在T2WI上均表現為局部皮層腫脹，呈高信號，其中3例DWI上出現皮層高信號，病灶不按腦血管分布。4例在MRA上出現病灶同側大腦中動脈血管較對側明顯增多。5例行增強掃描后均見病灶側腦膜明顯強化。5例患者臨床發作后均遺留有與MRI異常病灶相應的臨床神經功能缺失。其中3例病情穩定后復查MRI，發現異常病灶及MRA完全恢復正常。 結論 癲癇持續狀態引起腦部異常MRI表現特點：①常局限于大腦皮層的T2WI、DWI高信號，且病灶不按血管分布區分布；②MRA提示單側腦血管增多；③增強掃描見單側腦膜強化。大部分病灶可逆。這些特點可用來區別是癲癇引起的異常病灶還是腦部的致癇灶。【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.