Association between upper and lower respiratory disease among patients with primary ciliary dyskinesia: an international study.

Fecha de publicación: 01/03/2024 Fecha Ahead of Print: 04/03/2024

Autores de IIS La Fe

• Miguel Armengot Carceller

  Autor

• Ana Reula Martin

  Autor

Participantes ajenos a IIS La Fe

• Lam, Yin Ting
• Papon, Jean-Francois
• Alexandru, Mihaela
• Anagiotos, Andreas
• Boon, Mieke
• Burgess, Andrea
• Calmes, Doriane
• Crowley, Suzanne
• Dheyauldeen, Sinan Ahmed D
• Emiralioglu, Nagehan
• Erdem Eralp, Ela
• van Gogh, Christine
• Gokdemir, Yasemin
• Haarman, Eric G
• Harris, Amanda
• Hayn, Isolde
• Ismail-Koch, Hasnaa
• Karadag, Bulent
• Kempeneers, Celine
• Kieninger, Elisabeth
• Kim, Sookyung
• Lorent, Natalie
• Ozcelik, Ugur
• Pioch, Charlotte
• Raidt, Johanna
• Roehmel, Jobst
• Sperstad Kennelly, Synne
• Yiallouros, Panayiotis
• Goutaki, Myrofora
• EPIC-PCD team

Grupos

• Biomedicina Molecular, Celular y Genómica

  

  Leader

  José María Millán Salvador

Abstract

Introduction: Nearly all patients with primary ciliary dyskinesia (PCD) report ear-nose-throat (ENT) symptoms. However, scarce evidence exists about how ENT symptoms relate to pulmonary disease in PCD. We explored possible associations between upper and lower respiratory disease among patients with PCD in a multicentre study. Methods: We included patients from the ENT Prospective International Cohort (EPIC-PCD). We studied associations of several reported ENT symptoms and chronic rhinosinusitis (defined using patient-reported information and examination findings) with reported sputum production and shortness of breath, using ordinal logistic regression. In a subgroup with available lung function results, we used linear regression to study associations of chronic rhinosinusitis and forced expiratory volume in 1 s (FEV1) accounting for relevant factors. Results: We included 457 patients (median age 15 years, interquartile range 10-24 years; 54% males). Shortness of breath associated with reported nasal symptoms and ear pain of any frequency, often or daily hearing problems, headache when bending down (OR 2.1, 95% CI 1.29-3.54) and chronic rhinosinusitis (OR 2.3, 95% CI 1.57-3.38) regardless of polyp presence. Sputum production associated with daily reported nasal (OR 2.2, 95% CI 1.20-4.09) and hearing (OR 2.0, 95% CI 1.10-3.64) problems and chronic rhinosinusitis (OR 2.1, 95% CI 1.48-3.07). We did not find any association between chronic rhinosinusitis and FEV1. Conclusion: Reported upper airway symptoms and signs of chronic rhinosinusitis associated with reported pulmonary symptoms, but not with lung function. Our results emphasise the assessment and management of upper and lower respiratory disease as a common, interdependent entity among patients with PCD.

Copyright ©The authors 2024.

Keywords

• CHRONIC RHINOSINUSITIS; PREVALENCE; ASTHMA