Trajectory of Hyper IgE complications

Abstract Objective Our objective was to define the natural history of hyper IgE syndrome. Well, there have been a number of large cross-sectional studies. It has been difficult to provide anticipatory care for the growing adult population. Methods The USIDNET registry was used to identify patients with hyper IgE syndrome. We evaluated all patients regardless of genetic etiology. Complications were extracted as text from the problem list or ICD codes, and each complication was date stamped, which allowed us to estimate the age at which the complication arose. Results Many of the complications previously identified were seen in this cohort. The most feared complications were uncommon, however, developmental delay and psychosis affected nearly a fifth of the cohort which has not been previously reported. Conclusions As the number of adults with hyper IgE syndrome increases our awareness of late onset complications is important. Aspergillus and pneumatoceles were less common than seen in some other cohorts. However, the significant rate of psychosis will represent a significant challenge for clinicians.