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

Respir Med. 2014 Jun;108(6):931-4. doi: 10.1016/j.rmed.2014.03.009. Epub 2014 Apr 2.


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.

Keywords: Antibody; Cilia; Humoral immunodeficiency; Primary ciliary dyskinesia.

MeSH terms

  • Adolescent
  • Case-Control Studies
  • Child
  • Forced Expiratory Volume / physiology
  • Humans
  • Immunity, Humoral / physiology*
  • Immunologic Deficiency Syndromes / complications*
  • Immunologic Deficiency Syndromes / immunology
  • Immunologic Deficiency Syndromes / physiopathology
  • Kartagener Syndrome / complications*
  • Kartagener Syndrome / immunology
  • Kartagener Syndrome / physiopathology
  • Middle Aged
  • Retrospective Studies