Objective:Adult-onset Still's disease(AOSD)is a rare but clinically well-known polygenic systemic autoinflammatory disease.In this review,we aim to present frontiers in the pathogenesis,clinical features,diagnosis...Objective:Adult-onset Still's disease(AOSD)is a rare but clinically well-known polygenic systemic autoinflammatory disease.In this review,we aim to present frontiers in the pathogenesis,clinical features,diagnosis,biomarkers,disease course,prognosis,and treatment in AOSD.Data sources:We retrieved information from the PubMed database up to July 2019,using various search terms and relevant words,including AOSD and Still's disease.Study selection:We included data from peer-reviewed journals.Both basic and clinical studies were selected.Results:Pathogenesis of AOSD involves genetic background,infectious triggers,and immunopathogenesis,mainly the activation of macrophages and neutrophils followed by a cytokine storm.Diagnosis and prognosis evaluation of AOSD is still challenging;therefore,there is an urgent need to identify better biomarkers.Biologic agents,including interleukin(IL)-ip,IL-6,and tumor necrosis factor-a antagonists in the treatment of AOSD,have good prospect.Conclusion:This review highlights the advances in pathogenesis,potential biomarkers,disease course,and treatment in AOSD.展开更多
目的探讨商业化软件计算球后脂肪体积,分析其与甲状腺相关性眼病(TAO)进展及预后的关系。方法收集2016年1月~2016年12月在我院内分泌科确诊的35例(70个眼眶)TAO患者的临床资料。测量1.5T眼眶MRI球后脂肪体积及眼外肌信号强度比值(SIR)...目的探讨商业化软件计算球后脂肪体积,分析其与甲状腺相关性眼病(TAO)进展及预后的关系。方法收集2016年1月~2016年12月在我院内分泌科确诊的35例(70个眼眶)TAO患者的临床资料。测量1.5T眼眶MRI球后脂肪体积及眼外肌信号强度比值(SIR)分析其与临床各项指标的相关性,并收集12例(24个眼眶)健康人测量球后脂肪体积,初步比较TAO组及健康组体积的差异。结果脂肪体积与病程成正相关(r=0.480,P<0.01),病程6个月以内组与6~12个月组相比,脂肪体积差异不显著(P=0.084)。病程6个月以内组及病程6~12个月组球后脂肪体积均显著低于病程大于12个月组(P<0.01,P<0.05)。脂肪体积与突眼度存在相关性(r=0.622,P<0.01),突眼度每增加1 mm,球后脂肪体积将增加0.88 m L。临床活动性评分(CAS)与SIR值及促甲状腺素受体抗体(TRAb)存在相关性(r=0.536,r=0.416,P<0.01)。TAO组球后脂肪体积显著高于正常组(P<0.01)。结论 TAO病程1年以上可能是球后脂肪组织增多的高峰阶段,球后脂肪体积结合SIR值的测量有助于最佳激素治疗时机的探索及预后分析。展开更多
AIM To evaluate the role of albumin at the time of ulcerative colitis(UC) diagnosis in predicting the clinical course of disease.METHODS Nationwide cohort of patients with newly diagnosed UC in the Veterans Affairs he...AIM To evaluate the role of albumin at the time of ulcerative colitis(UC) diagnosis in predicting the clinical course of disease.METHODS Nationwide cohort of patients with newly diagnosed UC in the Veterans Affairs health care system was identified and divided into two categories: hypoalbuminemia(i.e.,≤ 3.5 gm/dl) or normal albumin levels(i.e.,> 3.5 gm/dl) at the time of UC diagnosis. The exposure of interest was presence of hypoalbuminemia defined asalbumin level ≤ 3.5 g/dl at the time of UC diagnosis. Patients were then followed over time to identify the use of ≥ 2 courses of corticosteroids(CS),thiopurines,anti-TNF medications and requirement of colectomy for UC management. RESULTS The eligible study cohort included 802 patients,but 92(11.4%) patients did not have their albumin levels checked at the time of UC diagnosis,and they were excluded. A total of 710 patients,who had albumin levels checked at time of UC diagnosis,were included in our study. Amongst them,536 patients had a normal albumin level and 174 patients had hypoalbuminemia. Patients with hypoalbuminemia at diagnosis had a higher likelihood of ≥ 2 courses of CS use(adjusted HR = 1.7,95%CI: 1.3-2.3),higher likelihood of thiopurine or anti-TNF use(adjusted HR = 1.72,95%CI: 1.23-2.40) than patients with normal albumin level at diagnosis. There was a trend of higher likelihood of colectomy in hypoalbuminemic patients,but it was not statistically significant(Adjusted HR = 1.7,95%CI: 0.90-3.25).CONCLUSION Hypoalbuminemia at disease diagnosis can serve as a prognostic marker to predict the clinical course of UC at the time of diagnosis.展开更多
AIM: To investigate patient-reported outcomes from, and adherence to, a low FODMAP diet among patients suffering from irritable bowel syndrome and inflammatory bowel disease.METHODS: Consecutive patients with irritabl...AIM: To investigate patient-reported outcomes from, and adherence to, a low FODMAP diet among patients suffering from irritable bowel syndrome and inflammatory bowel disease.METHODS: Consecutive patients with irritable bowel syndrome(IBS) or inflammatory bowel disease(IBD) and co-existing IBS fulfilling the ROME Ⅲ criteria, who previously attended an outpatient clinic for low FODMAP diet(LFD) dietary management and assessment by a gastroenterologist, were invited to participate in a retrospective questionnaire analysis. The questionnaires were sent and returned by regular mail and gathered information on recall of dietarytreatment, efficacy, symptoms, adherence, satisfaction, change in disease course and stool type, and quality of life. Before study enrolment all patients had to sign an informed written consent.RESULTS: One hundred and eighty patients were included, 131(73%) IBS and 49(27%) IBD patients. Median age was 43 years(range: 18-85) and 147(82%) were females. Median follow-up time was 16 mo(range: 2-80). Eighty-six percent reported either partial(54%) or full(32%) efficacy with greatest improvement of bloating(82%) and abdominal pain(71%). The proportion of patients with full efficacy tended to be greater in the IBD group than in the IBS group(42% vs 29%, P = 0.08). There was a significant reduction in patients with a chronic continuous disease course in both the IBS group(25%, P < 0.001) and IBD group(23%, P = 0.002) along with a significant increase in patients with a mild indolent disease course of 37%(P < 0.001) and 23%(P = 0.002), respectively. The proportion of patients having normal stools increased with 41% in the IBS group(P < 0.001) and 66% in the IBD group(P < 0.001). One-third of patients adhered to the diet and high adherence was associated with longer duration of dietary course(P < 0.001). Satisfaction with dietary management was seen in 83(70%) IBS patients and 24(55%) IBD patients. Eightyfour percent of patients lived on a modified LFD, where some foods rich in FODMAPs were reintroduced展开更多
基金This work was supported by grants from the National Natural Science Foundation of China(Nos.81671589,81871272).
文摘Objective:Adult-onset Still's disease(AOSD)is a rare but clinically well-known polygenic systemic autoinflammatory disease.In this review,we aim to present frontiers in the pathogenesis,clinical features,diagnosis,biomarkers,disease course,prognosis,and treatment in AOSD.Data sources:We retrieved information from the PubMed database up to July 2019,using various search terms and relevant words,including AOSD and Still's disease.Study selection:We included data from peer-reviewed journals.Both basic and clinical studies were selected.Results:Pathogenesis of AOSD involves genetic background,infectious triggers,and immunopathogenesis,mainly the activation of macrophages and neutrophils followed by a cytokine storm.Diagnosis and prognosis evaluation of AOSD is still challenging;therefore,there is an urgent need to identify better biomarkers.Biologic agents,including interleukin(IL)-ip,IL-6,and tumor necrosis factor-a antagonists in the treatment of AOSD,have good prospect.Conclusion:This review highlights the advances in pathogenesis,potential biomarkers,disease course,and treatment in AOSD.
文摘目的探讨商业化软件计算球后脂肪体积,分析其与甲状腺相关性眼病(TAO)进展及预后的关系。方法收集2016年1月~2016年12月在我院内分泌科确诊的35例(70个眼眶)TAO患者的临床资料。测量1.5T眼眶MRI球后脂肪体积及眼外肌信号强度比值(SIR)分析其与临床各项指标的相关性,并收集12例(24个眼眶)健康人测量球后脂肪体积,初步比较TAO组及健康组体积的差异。结果脂肪体积与病程成正相关(r=0.480,P<0.01),病程6个月以内组与6~12个月组相比,脂肪体积差异不显著(P=0.084)。病程6个月以内组及病程6~12个月组球后脂肪体积均显著低于病程大于12个月组(P<0.01,P<0.05)。脂肪体积与突眼度存在相关性(r=0.622,P<0.01),突眼度每增加1 mm,球后脂肪体积将增加0.88 m L。临床活动性评分(CAS)与SIR值及促甲状腺素受体抗体(TRAb)存在相关性(r=0.536,r=0.416,P<0.01)。TAO组球后脂肪体积显著高于正常组(P<0.01)。结论 TAO病程1年以上可能是球后脂肪组织增多的高峰阶段,球后脂肪体积结合SIR值的测量有助于最佳激素治疗时机的探索及预后分析。
文摘AIM To evaluate the role of albumin at the time of ulcerative colitis(UC) diagnosis in predicting the clinical course of disease.METHODS Nationwide cohort of patients with newly diagnosed UC in the Veterans Affairs health care system was identified and divided into two categories: hypoalbuminemia(i.e.,≤ 3.5 gm/dl) or normal albumin levels(i.e.,> 3.5 gm/dl) at the time of UC diagnosis. The exposure of interest was presence of hypoalbuminemia defined asalbumin level ≤ 3.5 g/dl at the time of UC diagnosis. Patients were then followed over time to identify the use of ≥ 2 courses of corticosteroids(CS),thiopurines,anti-TNF medications and requirement of colectomy for UC management. RESULTS The eligible study cohort included 802 patients,but 92(11.4%) patients did not have their albumin levels checked at the time of UC diagnosis,and they were excluded. A total of 710 patients,who had albumin levels checked at time of UC diagnosis,were included in our study. Amongst them,536 patients had a normal albumin level and 174 patients had hypoalbuminemia. Patients with hypoalbuminemia at diagnosis had a higher likelihood of ≥ 2 courses of CS use(adjusted HR = 1.7,95%CI: 1.3-2.3),higher likelihood of thiopurine or anti-TNF use(adjusted HR = 1.72,95%CI: 1.23-2.40) than patients with normal albumin level at diagnosis. There was a trend of higher likelihood of colectomy in hypoalbuminemic patients,but it was not statistically significant(Adjusted HR = 1.7,95%CI: 0.90-3.25).CONCLUSION Hypoalbuminemia at disease diagnosis can serve as a prognostic marker to predict the clinical course of UC at the time of diagnosis.
基金Supported by the Danish patient society of inflammatory bowel disease and irritable bowel syndrome patients,Colitis Crohn Foreningen
文摘AIM: To investigate patient-reported outcomes from, and adherence to, a low FODMAP diet among patients suffering from irritable bowel syndrome and inflammatory bowel disease.METHODS: Consecutive patients with irritable bowel syndrome(IBS) or inflammatory bowel disease(IBD) and co-existing IBS fulfilling the ROME Ⅲ criteria, who previously attended an outpatient clinic for low FODMAP diet(LFD) dietary management and assessment by a gastroenterologist, were invited to participate in a retrospective questionnaire analysis. The questionnaires were sent and returned by regular mail and gathered information on recall of dietarytreatment, efficacy, symptoms, adherence, satisfaction, change in disease course and stool type, and quality of life. Before study enrolment all patients had to sign an informed written consent.RESULTS: One hundred and eighty patients were included, 131(73%) IBS and 49(27%) IBD patients. Median age was 43 years(range: 18-85) and 147(82%) were females. Median follow-up time was 16 mo(range: 2-80). Eighty-six percent reported either partial(54%) or full(32%) efficacy with greatest improvement of bloating(82%) and abdominal pain(71%). The proportion of patients with full efficacy tended to be greater in the IBD group than in the IBS group(42% vs 29%, P = 0.08). There was a significant reduction in patients with a chronic continuous disease course in both the IBS group(25%, P < 0.001) and IBD group(23%, P = 0.002) along with a significant increase in patients with a mild indolent disease course of 37%(P < 0.001) and 23%(P = 0.002), respectively. The proportion of patients having normal stools increased with 41% in the IBS group(P < 0.001) and 66% in the IBD group(P < 0.001). One-third of patients adhered to the diet and high adherence was associated with longer duration of dietary course(P < 0.001). Satisfaction with dietary management was seen in 83(70%) IBS patients and 24(55%) IBD patients. Eightyfour percent of patients lived on a modified LFD, where some foods rich in FODMAPs were reintroduced
