Primary ciliary dyskinesia and humoral immunodeficiency – Is there a missing link?

BackgroundPrimary ciliary dyskinesia (PCD) and humoral mmunodeficiency (HID) are both rare disorders which cause recurrent upper and lower respiratory tract infections.ObjectiveTo examine the concurrence of PCD and HID in a patient cohort with known PCD.MethodsRetrospective review of the patient files.ResultsWe describe 11 patients of a cohort of 168 patients with PCD (6.5%) with a combination of PCD and some form of HID. The patients all presented with typical clinical symptoms for PCD, however the role of the concomitant immunological abnormalities is not clear.ConclusionPCD and HID coincided in 6.5% of the patients. We suggest that a common pathophysiological pathway results in both disorders.