Variations in Using Diagnosis Codes for Defining Age-Related Macular Degeneration Cohorts

Data harmonization is vital for secondary electronic health record data analysis, especially when combining data from multiple sources. Currently, there is a gap in knowledge as to how studies identify cohorts of patients with age-related macular degeneration (AMD), a leading cause of blindness. We hypothesize that there is variation in using medical condition codes to define cohorts of AMD patients that can lead to either the under- or overrepresentation of such cohorts. This study identified articles studying AMD using the International Classification of Diseases (ICD-9, ICD-9-CM, ICD-10, and ICD-10-CM). The data elements reviewed included the year of publication; dataset origin (Veterans Affairs, registry, national or commercial claims database, and institutional EHR); total number of subjects; and ICD codes used. A total of thirty-seven articles were reviewed. Six (16%) articles used cohort definitions from two ICD terminologies. The Medicare database was the most used dataset (14, 38%), and there was a noted increase in the use of other datasets in the last few years. We identified substantial variation in the use of ICD codes for AMD. For the studies that used ICD-10 terminologies, 7 (out of 9, 78%) defined the AMD codes correctly, whereas, for the studies that used ICD-9 and 9-CM terminologies, only 2 (out of 30, 7%) defined and utilized the appropriate AMD codes (p = 0.0001). Of the 43 cohort definitions used from 37 articles, 31 (72%) had missing or incomplete AMD codes used, and only 9 (21%) used the exact codes. Additionally, 13 articles (35%) captured ICD codes that were not within the scope of AMD diagnosis. Efforts to standardize data are needed to provide a reproducible research output.