San Raffaele Diabetes Research Institute, IRCCS San Raffaele Hospital, Milan, Italy
JDRF, New York, NY.
Diabetes Care. 2020 Jan;43(1):5-12. doi: 10.2337/dc19-0880. Epub 2019 Nov 21.
The clinical diagnosis of new-onset type 1 diabetes has, for many years, been considered relatively straightforward. Recently, however, there is increasing awareness that within this single clinical phenotype exists considerable heterogeneity: disease onset spans the complete age range; genetic susceptibility is complex; rates of progression differ markedly, as does insulin secretory capacity; and complication rates, glycemic control, and therapeutic intervention efficacy vary widely. Mechanistic and immunopathological studies typically show considerable patchiness across subjects, undermining conclusions regarding disease pathways. Without better understanding, type 1 diabetes heterogeneity represents a major barrier both to deciphering pathogenesis and to the translational effort of designing, conducting, and interpreting clinical trials of disease-modifying agents. This realization comes during a period of unprecedented change in clinical medicine, with increasing emphasis on greater individualization and precision. For complex disorders such as type 1 diabetes, the option of maintaining the "single disease" approach appears untenable, as does the notion of individualizing each single patient's care, obliging us to conceptualize type 1 diabetes less in terms of phenotypes (observable characteristics) and more in terms of disease endotypes (underlying biological mechanisms). Here, we provide our view on an approach to dissect heterogeneity in type 1 diabetes. Using lessons from other diseases and the data gathered to date, we aim to delineate a roadmap through which the field can incorporate the endotype concept into laboratory and clinical practice. We predict that such an effort will accelerate the implementation of precision medicine and has the potential for impact on our approach to translational research, trial design, and clinical management.
新诊断 1 型糖尿病的临床诊断多年来一直被认为相对简单。然而,最近人们越来越意识到,在这种单一的临床表型中存在着相当大的异质性:疾病的发病范围涵盖了整个年龄范围;遗传易感性复杂;进展速度差异显著,胰岛素分泌能力也不同;并发症发生率、血糖控制和治疗干预效果差异很大。机制和免疫病理研究通常在不同的个体中显示出相当大的差异,这破坏了对疾病途径的结论。如果没有更好的理解,1 型糖尿病的异质性不仅是解析发病机制的主要障碍,也是设计、进行和解释疾病修饰剂临床试验的转化努力的主要障碍。这种认识是在临床医学发生前所未有的变化时期提出的,越来越强调更大的个体化和精确化。对于 1 型糖尿病等复杂疾病,维持“单一疾病”方法的选择似乎是不可行的,个体化每个患者的护理的概念也是不可行的,迫使我们从疾病内型(可观察到的特征)的角度来考虑 1 型糖尿病,而不是从表型(可观察到的特征)的角度。在这里,我们提供了一种分析 1 型糖尿病异质性的方法。利用其他疾病的经验教训和迄今为止收集的数据,我们旨在制定一条路线图,使该领域能够将内型概念纳入实验室和临床实践。我们预测,这种努力将加速精准医学的实施,并有可能影响我们对转化研究、试验设计和临床管理的方法。