Background: Hereditary angioedema (HAE) is a rare, autosomal dominant genetic disorder characterized by sporadic episodes of swelling. The prevalence of HAE is difficult to quantify using large databases of commercially insured patients due to misclassification resulting from non-specific diagnostic coding and prohibited access to patient medical records for case confirmation.
Objectives: To develop Health Insurance Portability and Accountability Act (HIPAA)-compliant patient summary reports (PSRs) that can be shared with Angioedema Center (AEC) physicians specializing in the treatment of HAE for review and case adjudication.
Methods: Patients with at least one medical claim with ICD-10-CM diagnosis code D84.1 (defects in the complement system), or T78.3* (angioneurotic edema); and at least one pharmacy claim for medication indicated for HAE were selected from the IQVIA PharMetrics® Plus database for AEC physician case adjudication. An anonymized PSR was programmatically generated, with results outputted to Excel, for each potential HAE patient identified by the algorithm (i.e., one patient per worksheet). Each worksheet contained chronologically assembled summary counts of relevant diagnoses, medications dispensed (including clinician specialty and setting), and complement C4 / C1 esterase inhibitor lab tests ordered. Summary counts were provided by month of observation. Each patient contributed between 2 – 5 years of data between January 1, 2016 and December 31, 2020. The PSRs will be reviewed and adjudicated for HAE case status by 3 AEC physicians. Final HAE case status based on AEC physician consensus will be: 1) Likely HAE, 2) Not Likely HAE, or 3) Cannot assess due to insufficient data.
Results: There were 157 patients identified by the algorithm and for whom PSRs were generated. A pilot of 5 PSRs was conducted among the 3 AEC physicians and agreement on HAE case status was achieved for all 5 PSRs based on consensus. The remaining 152 PSRs are currently under review by the 3 AEC physicians and results are expected by March 2023.
Conclusions: Patient summary reports provide a viable HIPAA-compliant and cost-effective alternative for case adjudication in circumstances where access to patient medical records is not possible or is prohibitively expensive.
