MOTIVATION: Editome Disease Knowledgebase (EDK) is a curated resource of knowledge between RNA editome and human diseases. Since its first release in 2018, a number of studies have discovered previously uncharacterized editome-disease associations and generated an abundance of RNA editing datasets. Thus, it is desirable to make significant updates for EDK by incorporating more editome-disease associations as well as their related editing profiles. RESULTS: Here, we present EDK v2.0, an updated version of editome-disease associations based on both literature curation and integrative analysis. EDK v2.0 incorporates a curated collection of 1097 editome-disease associations involving 115 diseases from 321 publications. Meanwhile, based on a standardized pipeline, EDK v2.0 provides RNA editing profiles from 48 datasets covering 2536 samples across 55 diseases. Through differential analysis on RNA editing, it further identifies a total of 7190 differential edited genes and 86 242 differential editing sites (DESs), leading to 266 339 DES-disease associations. Moreover, a curated list of 28 160 AVAILABILITY AND IMPLEMENTATION: https://ngdc.cncb.ac.cn/edk/.