Primary ciliary dyskinesia and humoral immunodeficiency--is there a missing link?

BACKGROUND

Primary ciliary dyskinesia (PCD) and humoral mmunodeficiency (HID) are both rare disorders which cause recurrent upper and lower respiratory tract infections.

OBJECTIVE

To examine the concurrence of PCD and HID in a patient cohort with known PCD.

METHODS

Retrospective review of the patient files.

RESULTS

We 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.

CONCLUSION

PCD and HID coincided in 6.5% of the patients. We suggest that a common pathophysiological pathway results in both disorders.