Copyright ©The Author(s) 2017. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Pediatr. Aug 8, 2017; 6(3): 132-142
Published online Aug 8, 2017. doi: 10.5409/wjcp.v6.i3.132
Role of vitamin D in cystic fibrosis and non-cystic fibrosis bronchiectasis
Maria Moustaki, Ioanna Loukou, Kostas N Priftis, Konstantinos Douros
Maria Moustaki, Ioanna Loukou, Cystic Fibrosis Unit, “Aghia Sophia” Children’s Hospital, 11527 Athens, Greece
Kostas N Priftis, Konstantinos Douros, Pediatric Allergy and Respiratory Unit, 3rd Department of Pediatrics, “Attikon” Hospital, University of Athens School of Medicine, 12462 Athens, Greece
Author contributions: Moustaki M and Loukou I wrote the first draft of the manuscript; Priftis KN and Douros K reviewed the draft, made changes, and completed the final version; all authors approved the submitted version.
Conflict-of-interest statement: The authors declare no conflict of interest.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: Dr. Kostas N Priftis, Assistant Professor, Pediatric Allergy and Respiratory Unit, 3rd Department of Pediatrics, “Attikon” Hospital, University of Athens School of Medicine, 1 Rimini Str, 12462 Chaidari, Greece.
Telephone: +30-210-5832228 Fax: +30-210-5832229
Received: January 11, 2017
Peer-review started: January 11, 2017
First decision: February 20, 2017
Revised: May 26, 2017
Accepted: June 12, 2017
Article in press: June 13, 2017
Published online: August 8, 2017

Bronchiectasis is usually classified as cystic fibrosis (CF) related or CF unrelated (non-CF); the latter is not considered an orphan disease any more, even in developed countries. Irrespective of the underlying etiology, bronchiectasis is the result of interaction between host, pathogens, and environment. Vitamin D is known to be involved in a wide spectrum of significant immunomodulatory effects such as down-regulation of pro-inflammatory cytokines and chemokines. Respiratory epithelial cells constitutively express 1α-hydroxylase leading to the local transformation of the inactive 25(OH)-vitamin D to the active 1,25(OH)2-vitamin D. The latter through its autocrine and paracrine functions up-regulates vitamin D dependent genes with important consequences in the local immunity of lungs. Despite the scarcity of direct evidence on the involvement of vitamin D deficiency states in the development of bronchiectasis in either CF or non-CF patients, it is reasonable to postulate that vitamin D may play some role in the pathogenesis of lung diseases and especially bronchiectasis. The potential contribution of vitamin D deficiency in the process of bronchiectasis is of particular clinical importance, taking into consideration the increasing prevalence of vitamin D deficiency worldwide and the significant morbidity of bronchiectasis. Given the well-established association of vitamin D deficiency with increased inflammation, and the indicative evidence for harmful consequences in lungs, it is intriguing to speculate that the administration of vitamin D supplementation could be a reasonable and cost effective supplementary therapeutic approach for children with non-CF bronchiectasis. Regarding CF patients, maybe in the future as more data become available, we have to re-evaluate our policy on the most appropriate dosage scheme for vitamin D.

Keywords: Vitamin D, Bronchiectasis, Cystic fibrosis, Vitamin D supplementation

Core tip: Vitamin D deficiency seems to be associated with respiratory health. Herein, we present the experimental and epidemiological data that imply a role of vitamin D deficiency in the development of cystic fibrosis (CF) and non-CF bronchiectasis. Numerous experimental data provide insight to the mechanism by which vitamin D modulates immunity, and therefore its deficiency may enhance the susceptibility to infectious diseases and affect the control of inflammation process. Epidemiological data provide evidence for the association of vitamin D deficiency and bronchiectasis, either directly or indirectly, through its relation with the risk for respiratory tract infections. This knowledge is of interest for the pediatrician as vitamin D supplementation may be a future candidate therapeutic option for bronchiectasis.