First Department of Medicine, Semmelweis University, H-1083 Budapest, Hungary.
World J Gastroenterol. 2013;19(14):2217-26. doi: 10.3748/wjg.v19.i14.2217.
To investigate the evolution of disease phenotype in adult and pediatric onset Crohn's disease (CD) populations, diagnosed between 1977 and 2008.
Data of 506 incident CD patients were analyzed (age at diagnosis: 28.5 years, interquartile range: 22-38 years). Both in- and outpatient records were collected prospectively with a complete clinical follow-up and comprehensively reviewed in the population-based Veszprem province database, which included incident patients diagnosed between January 1, 1977 and December 31, 2008 in adult and pediatric onset CD populations. Disease phenotype according to the Montreal classification and long-term disease course was analysed according to the age at onset in time-dependent univariate and multivariate analysis.
Among this population-based cohort, seventy-four (12.8%) pediatric-onset CD patients were identified (diagnosed ≤ 17 years of age). There was no significant difference in the distribution of disease behavior between pediatric (B1: 62%, B2: 15%, B3: 23%) and adult-onset CD patients (B1: 56%, B2: 21%, B3: 23%) at diagnosis, or during follow-up. Overall, the probability of developing complicated disease behaviour was 49.7% and 61.3% in the pediatric and 55.1% and 62.4% in the adult onset patients after 5- and 10-years of follow-up. Similarly, time to change in disease behaviour from non stricturing, non penetrating (B1) to complicated, stricturing or penetrating (B2/B3) disease was not significantly different between pediatric and adult onset CD in a Kaplan-Meier analysis. Calendar year of diagnosis (P = 0.04), ileal location (P < 0.001), perianal disease (P < 0.001), smoking (P = 0.038) and need for steroids (P < 0.001) were associated with presence of, or progression to, complicated disease behavior at diagnosis and during follow-up. A change in disease location was observed in 8.9% of patients and it was associated with smoking status (P = 0.01), but not with age at diagnosis.
Long-term evolution of disease behavior was not different in pediatric- and adult-onset CD patients in this population-based cohort but was associated to location, perianal disease and smoking status.
研究 1977 年至 2008 年间诊断的成人和儿童发病克罗恩病(CD)患者疾病表型的演变。
分析了 506 例新发 CD 患者的数据(诊断时年龄:28.5 岁,四分位距:22-38 岁)。采用前瞻性方法收集门诊和住院记录,对基于人群的维斯普雷姆省数据库进行全面临床随访和综合回顾,该数据库包括 1977 年 1 月 1 日至 2008 年 12 月 31 日期间诊断的成人和儿童发病 CD 患者。根据蒙特利尔分类和长期疾病过程,根据发病年龄,在时间依赖性单变量和多变量分析中分析疾病表型。
在这个基于人群的队列中,确定了 74 例(12.8%)儿童发病 CD 患者(诊断年龄≤17 岁)。在诊断时或随访期间,儿科(B1:62%,B2:15%,B3:23%)和成人发病 CD 患者(B1:56%,B2:21%,B3:23%)之间,疾病行为的分布无显著差异。总体而言,在 5 年和 10 年的随访后,儿科和成人发病患者中出现复杂疾病行为的概率分别为 49.7%和 61.3%,5-和 10 年的随访后,儿科和成人发病患者中出现复杂疾病行为的概率分别为 49.7%和 61.3%。同样,在 Kaplan-Meier 分析中,儿童和成人发病 CD 患者从非狭窄、非穿透(B1)疾病到复杂、狭窄或穿透(B2/B3)疾病的疾病行为改变时间也无显著差异。诊断年份(P=0.04)、回肠部位(P<0.001)、肛周疾病(P<0.001)、吸烟(P=0.038)和需要类固醇(P<0.001)与诊断时和随访期间的复杂疾病行为的存在或进展相关。8.9%的患者发生了疾病部位的改变,与吸烟状态有关(P=0.01),但与诊断年龄无关。
在这个基于人群的队列中,儿童发病和成人发病 CD 患者的疾病行为演变长期无差异,但与部位、肛周疾病和吸烟状态有关。