{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T21:12:00Z","timestamp":1762031520727,"version":"build-2065373602"},"reference-count":122,"publisher":"Elsevier BV","issue":"6","license":[{"start":{"date-parts":[[2023,11,1]],"date-time":"2023-11-01T00:00:00Z","timestamp":1698796800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2023,11,1]],"date-time":"2023-11-01T00:00:00Z","timestamp":1698796800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T00:00:00Z","timestamp":1761955200000},"content-version":"vor","delay-in-days":731,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","cysticfibrosisjournal.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Cystic Fibrosis"],"published-print":{"date-parts":[[2023,11]]},"DOI":"10.1016\/j.jcf.2023.03.007","type":"journal-article","created":{"date-parts":[[2023,3,23]],"date-time":"2023-03-23T18:49:59Z","timestamp":1679597399000},"page":"969-979","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":3,"title":["Autoimmunity in people with cystic fibrosis"],"prefix":"10.1016","volume":"22","author":[{"given":"Christina","family":"Chadwick","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7553-9751","authenticated-orcid":false,"given":"Heather","family":"Lehman","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0979-3057","authenticated-orcid":false,"given":"Shelby","family":"Luebbert","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3587-7916","authenticated-orcid":false,"given":"Rabheh","family":"Abdul-Aziz","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3685-4078","authenticated-orcid":false,"given":"Drucy","family":"Borowitz","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"year":"2021","series-title":"Patient registry 2020 annual data report","key":"10.1016\/j.jcf.2023.03.007_bib0001"},{"issue":"12","key":"10.1016\/j.jcf.2023.03.007_bib0002","doi-asserted-by":"crossref","first-page":"3595","DOI":"10.1002\/ppul.25627","article-title":"Review of CFTR modulators 2020","volume":"56","author":"Goetz","year":"2021","journal-title":"Pediatr Pulmonol"},{"issue":"10212","key":"10.1016\/j.jcf.2023.03.007_bib0003","doi-asserted-by":"crossref","first-page":"1940","DOI":"10.1016\/S0140-6736(19)32597-8","article-title":"Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial","volume":"394","author":"Heijerman","year":"2019","journal-title":"Lancet"},{"issue":"19","key":"10.1016\/j.jcf.2023.03.007_bib0004","doi-asserted-by":"crossref","first-page":"1809","DOI":"10.1056\/NEJMoa1908639","article-title":"Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele","volume":"381","author":"Middleton","year":"2019","journal-title":"N Engl J Med"},{"key":"10.1016\/j.jcf.2023.03.007_bib0005","article-title":"Epigenetics and autoimmune diseases","volume":"2012","author":"Quintero-Ronderos","year":"2012","journal-title":"Autoimmune Dis"},{"issue":"4","key":"10.1016\/j.jcf.2023.03.007_bib0006","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1038\/s41577-020-00454-2","article-title":"Rethinking peripheral T cell tolerance: checkpoints across a T cell's journey","volume":"21","author":"ElTanbouly","year":"2021","journal-title":"Nat Rev Immunol"},{"issue":"1","key":"10.1016\/j.jcf.2023.03.007_bib0007","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1016\/j.clim.2012.10.005","article-title":"The role of B lymphocytes in the progression from autoimmunity to autoimmune disease","volume":"146","author":"Salinas","year":"2013","journal-title":"Clin Immunol"},{"issue":"1","key":"10.1016\/j.jcf.2023.03.007_bib0008","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1097\/01.mpg.0000166801.61708.60","article-title":"Frequency of ASCA seropositivity in children with cystic fibrosis","volume":"41","author":"Condino","year":"2005","journal-title":"J Pediatr Gastroenterol Nutr"},{"issue":"12","key":"10.1016\/j.jcf.2023.03.007_bib0009","doi-asserted-by":"crossref","first-page":"987","DOI":"10.1007\/s004310050983","article-title":"Antineutrophil cytoplasmic antibodies in children","volume":"157","author":"Sediva","year":"1998","journal-title":"Eur J Pediatr"},{"issue":"8","key":"10.1016\/j.jcf.2023.03.007_bib0010","doi-asserted-by":"crossref","first-page":"476","DOI":"10.1080\/08916934.2020.1839890","article-title":"IgA autoantibodies directed against self DNA are elevated in cystic fibrosis and associated with more severe lung dysfunction","volume":"53","author":"Yadav","year":"2020","journal-title":"Autoimmunity"},{"issue":"11","key":"10.1016\/j.jcf.2023.03.007_bib0011","doi-asserted-by":"crossref","first-page":"1160","DOI":"10.1016\/j.autrev.2017.09.012","article-title":"Neutrophil extracellular traps (NETs) in autoimmune diseases: a comprehensive review","volume":"16","author":"Lee","year":"2017","journal-title":"Autoimmun Rev"},{"issue":"1","key":"10.1016\/j.jcf.2023.03.007_bib0012","doi-asserted-by":"crossref","first-page":"L71","DOI":"10.1152\/ajplung.2001.281.1.L71","article-title":"Activation of NF-kappaB in airway epithelial cells is dependent on CFTR trafficking and Cl- channel function","volume":"281","author":"Weber","year":"2001","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"issue":"3","key":"10.1016\/j.jcf.2023.03.007_bib0013","doi-asserted-by":"crossref","first-page":"396","DOI":"10.1165\/ajrcmb.23.3.3949","article-title":"Exaggerated activation of nuclear factor-kappaB and altered IkappaB-beta processing in cystic fibrosis bronchial epithelial cells","volume":"23","author":"Venkatakrishnan","year":"2000","journal-title":"Am J Respir Cell Mol Biol"},{"issue":"2","key":"10.1016\/j.jcf.2023.03.007_bib0014","doi-asserted-by":"crossref","first-page":"L383","DOI":"10.1152\/ajplung.00403.2005","article-title":"CFTR inhibition mimics the cystic fibrosis inflammatory profile","volume":"292","author":"Perez","year":"2007","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"issue":"4","key":"10.1016\/j.jcf.2023.03.007_bib0015","first-page":"1075","article-title":"Riches DW. Early pulmonary inflammation in infants with cystic fibrosis","volume":"151","author":"Khan","year":"1995","journal-title":"Am J Respir Crit Care Med"},{"issue":"5","key":"10.1016\/j.jcf.2023.03.007_bib0016","doi-asserted-by":"crossref","first-page":"1298","DOI":"10.1093\/hmg\/ddt520","article-title":"Coregulation and modulation of NFkappaB-related genes in celiac disease: uncovered aspects of gut mucosal inflammation","volume":"23","author":"Fernandez-Jimenez","year":"2014","journal-title":"Hum Mol Genet"},{"issue":"5663","key":"10.1016\/j.jcf.2023.03.007_bib0017","doi-asserted-by":"crossref","first-page":"1532","DOI":"10.1126\/science.1092385","article-title":"Neutrophil extracellular traps kill bacteria","volume":"303","author":"Brinkmann","year":"2004","journal-title":"Science"},{"issue":"8","key":"10.1016\/j.jcf.2023.03.007_bib0018","doi-asserted-by":"crossref","first-page":"981","DOI":"10.1038\/nm1612","article-title":"DNase Sda1 provides selection pressure for a switch to invasive group A streptococcal infection","volume":"13","author":"Walker","year":"2007","journal-title":"Nat Med"},{"issue":"4","key":"10.1016\/j.jcf.2023.03.007_bib0019","doi-asserted-by":"crossref","first-page":"668","DOI":"10.1111\/j.1462-5822.2005.00659.x","article-title":"Neutrophil extracellular traps capture and kill Candida albicans yeast and hyphal forms","volume":"8","author":"Urban","year":"2006","journal-title":"Cell Microbiol"},{"issue":"2","key":"10.1016\/j.jcf.2023.03.007_bib0020","doi-asserted-by":"crossref","first-page":"e32366","DOI":"10.1371\/journal.pone.0032366","article-title":"Neutrophil extracellular traps directly induce epithelial and endothelial cell death: a predominant role of histones","volume":"7","author":"Saffarzadeh","year":"2012","journal-title":"PLoS ONE"},{"key":"10.1016\/j.jcf.2023.03.007_bib0021","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1186\/s12950-017-0176-1","article-title":"Neutrophil extracellular traps and the dysfunctional innate immune response of cystic fibrosis lung disease: a review","volume":"14","author":"Law","year":"2017","journal-title":"J Inflamm (Lond)."},{"issue":"9","key":"10.1016\/j.jcf.2023.03.007_bib0022","doi-asserted-by":"crossref","first-page":"e23637","DOI":"10.1371\/journal.pone.0023637","article-title":"Neutrophil extracellular trap (NET)-mediated killing of Pseudomonas aeruginosa: evidence of acquired resistance within the CF airway, independent of CFTR","volume":"6","author":"Young","year":"2011","journal-title":"PLoS ONE"},{"issue":"3","key":"10.1016\/j.jcf.2023.03.007_bib0023","doi-asserted-by":"crossref","first-page":"260","DOI":"10.1159\/000295791","article-title":"In cystic fibrosis homozygotes and heterozygotes, neutrophil apoptosis is delayed and modulated by diamide or roscovitine: evidence for an innate neutrophil disturbance","volume":"2","author":"Moriceau","year":"2010","journal-title":"J Innate Immun"},{"issue":"2","key":"10.1016\/j.jcf.2023.03.007_bib0024","doi-asserted-by":"crossref","first-page":"134","DOI":"10.1136\/thoraxjnl-2017-210134","article-title":"Delayed neutrophil apoptosis enhances NET formation in cystic fibrosis","volume":"73","author":"Gray","year":"2018","journal-title":"Thorax"},{"issue":"1","key":"10.1016\/j.jcf.2023.03.007_bib0025","doi-asserted-by":"crossref","first-page":"L31","DOI":"10.1152\/ajplung.2001.281.1.L31","article-title":"Antioxidant imbalance in the lungs of cystic fibrosis transmembrane conductance regulator protein mutant mice","volume":"281","author":"Velsor","year":"2001","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"issue":"6","key":"10.1016\/j.jcf.2023.03.007_bib0026","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1164\/rccm.201206-1150OC","article-title":"A Th17- and Th2-skewed cytokine profile in cystic fibrosis lungs represents a potential risk factor for Pseudomonas aeruginosa infection","volume":"187","author":"Tiringer","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"issue":"6","key":"10.1016\/j.jcf.2023.03.007_bib0027","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1164\/rccm.201207-1346OC","article-title":"Th17\/Treg imbalance in murine cystic fibrosis is linked to indoleamine 2,3-dioxygenase deficiency but corrected by kynurenines","volume":"187","author":"Iannitti","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"issue":"8","key":"10.1016\/j.jcf.2023.03.007_bib0028","doi-asserted-by":"crossref","first-page":"914","DOI":"10.1164\/rccm.201407-1381OC","article-title":"Regulatory T-cell impairment in cystic fibrosis patients with chronic pseudomonas infection","volume":"191","author":"Hector","year":"2015","journal-title":"Am J Respir Crit Care Med"},{"issue":"S3","key":"10.1016\/j.jcf.2023.03.007_bib0029","doi-asserted-by":"crossref","first-page":"S30","DOI":"10.1002\/ppul.24129","article-title":"Inflammation in cystic fibrosis: an update","volume":"53","author":"Roesch","year":"2018","journal-title":"Pediatr Pulmonol"},{"issue":"5","key":"10.1016\/j.jcf.2023.03.007_bib0030","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1056\/NEJM198001313020501","article-title":"Hypogammaglobulinemia in patients with cystic fibrosis","volume":"302","author":"Matthews","year":"1980","journal-title":"N Engl J Med"},{"issue":"5","key":"10.1016\/j.jcf.2023.03.007_bib0031","doi-asserted-by":"crossref","first-page":"695","DOI":"10.1016\/S0022-3476(84)80946-4","article-title":"Progression of cystic fibrosis lung disease as a function of serum immunoglobulin G levels: a 5-year longitudinal study","volume":"104","author":"Wheeler","year":"1984","journal-title":"J Pediatr"},{"issue":"2","key":"10.1016\/j.jcf.2023.03.007_bib0032","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1002\/ppul.20050","article-title":"Immunoglobulin and IgG subclass levels in a regional pediatric cystic fibrosis clinic","volume":"39","author":"Garside","year":"2005","journal-title":"Pediatr Pulmonol"},{"issue":"2","key":"10.1016\/j.jcf.2023.03.007_bib0033","doi-asserted-by":"crossref","first-page":"128","DOI":"10.1016\/j.jcf.2010.12.004","article-title":"Change in IgG and evolution of lung function in children with cystic fibrosis","volume":"10","author":"Proesmans","year":"2011","journal-title":"J Cyst Fibros"},{"key":"10.1016\/j.jcf.2023.03.007_bib0034","doi-asserted-by":"crossref","first-page":"S257","DOI":"10.1016\/S1569-1993(22)01138-9","article-title":"Pathological autoimmune responses in cystic fibrosis exacerbation","volume":"21","author":"Margaroli","year":"2022","journal-title":"J Cyst Fibros"},{"key":"10.1016\/j.jcf.2023.03.007_bib0035","doi-asserted-by":"crossref","DOI":"10.1016\/j.ebiom.2020.102974","article-title":"Lung immunoglobulin A immunity dysregulation in cystic fibrosis","volume":"60","author":"Collin","year":"2020","journal-title":"EBioMedicine"},{"issue":"4","key":"10.1016\/j.jcf.2023.03.007_bib0036","doi-asserted-by":"crossref","DOI":"10.1183\/13993003.01873-2016","article-title":"Bacteria-driven peribronchial lymphoid neogenesis in bronchiectasis and cystic fibrosis","volume":"49","author":"Frija-Masson","year":"2017","journal-title":"Eur Respir J"},{"issue":"7405","key":"10.1016\/j.jcf.2023.03.007_bib0037","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1038\/nature11130","article-title":"Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung","volume":"487","author":"Pezzulo","year":"2012","journal-title":"Nature"},{"issue":"4","key":"10.1016\/j.jcf.2023.03.007_bib0038","doi-asserted-by":"crossref","first-page":"402","DOI":"10.1002\/ppul.21028","article-title":"Relationship between antimicrobial proteins and airway inflammation and infection in cystic fibrosis","volume":"44","author":"Sagel","year":"2009","journal-title":"Pediatr Pulmonol"},{"issue":"10","key":"10.1016\/j.jcf.2023.03.007_bib0039","doi-asserted-by":"crossref","first-page":"2946","DOI":"10.1007\/s10620-015-3842-2","article-title":"Fecal human beta-Defensin 2 in children with cystic fibrosis: is There a diminished intestinal innate immune response?","volume":"60","author":"Ooi","year":"2015","journal-title":"Dig Dis Sci"},{"issue":"3","key":"10.1016\/j.jcf.2023.03.007_bib0040","doi-asserted-by":"crossref","first-page":"304","DOI":"10.1097\/MPG.0b013e3181d1b013","article-title":"Evidence of intestinal inflammation in patients with cystic fibrosis","volume":"51","author":"Werlin","year":"2010","journal-title":"J Pediatr Gastroenterol Nutr"},{"key":"10.1016\/j.jcf.2023.03.007_bib0041","doi-asserted-by":"crossref","DOI":"10.1155\/2012\/948367","article-title":"Update of faecal markers of inflammation in children with cystic fibrosis","volume":"2012","author":"Lee","year":"2012","journal-title":"Mediators Inflamm"},{"issue":"4","key":"10.1016\/j.jcf.2023.03.007_bib0042","doi-asserted-by":"crossref","DOI":"10.1128\/mBio.00251-12","article-title":"Serial analysis of the gut and respiratory microbiome in cystic fibrosis in infancy: interaction between intestinal and respiratory tracts and impact of nutritional exposures","volume":"3","author":"Madan","year":"2012","journal-title":"MBio"},{"issue":"4","key":"10.1016\/j.jcf.2023.03.007_bib0043","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1111\/joim.12395","article-title":"Human autoimmune diseases: a comprehensive update","volume":"278","author":"Wang","year":"2015","journal-title":"J Intern Med"},{"issue":"2","key":"10.1016\/j.jcf.2023.03.007_bib0044","doi-asserted-by":"crossref","first-page":"396","DOI":"10.1183\/09031936.00069007","article-title":"Mucosal inflammation in idiopathic bronchiectasis: cellular and molecular mechanisms","volume":"31","author":"Fuschillo","year":"2008","journal-title":"Eur Respir J"},{"issue":"135","key":"10.1016\/j.jcf.2023.03.007_bib0045","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1183\/09059180.00008014","article-title":"Rheumatoid arthritis-associated lung disease","volume":"24","author":"Shaw","year":"2015","journal-title":"Eur Respir Rev"},{"issue":"6","key":"10.1016\/j.jcf.2023.03.007_bib0046","doi-asserted-by":"crossref","first-page":"845","DOI":"10.1097\/MPA.0000000000001587","article-title":"Measures of Dietary Fat and Energy Absorption in Healthy Adults","volume":"49","author":"Bashaw","year":"2020","journal-title":"Pancreas"},{"issue":"4","key":"10.1016\/j.jcf.2023.03.007_bib0047","doi-asserted-by":"crossref","first-page":"518","DOI":"10.1016\/j.immuni.2014.09.008","article-title":"HIF transcription factors, inflammation, and immunity","volume":"41","author":"Palazon","year":"2014","journal-title":"Immunity"},{"issue":"2","key":"10.1016\/j.jcf.2023.03.007_bib0048","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1016\/j.jcmgh.2019.04.013","article-title":"Pathophysiology of cystic fibrosis liver disease: a channelopathy leading to alterations in innate immunity and in microbiota","volume":"8","author":"Fiorotto","year":"2019","journal-title":"Cell Mol Gastroenterol Hepatol"},{"issue":"6","key":"10.1016\/j.jcf.2023.03.007_bib0049","doi-asserted-by":"crossref","first-page":"518","DOI":"10.1177\/0961203313501401","article-title":"Diet, microbiota and autoimmune diseases","volume":"23","author":"Vieira","year":"2014","journal-title":"Lupus"},{"issue":"22","key":"10.1016\/j.jcf.2023.03.007_bib0050","doi-asserted-by":"crossref","first-page":"8015","DOI":"10.1128\/AEM.05933-11","article-title":"Cross-sectional and longitudinal comparisons of the predominant fecal microbiota compositions of a group of pediatric patients with cystic fibrosis and their healthy siblings","volume":"77","author":"Duytschaever","year":"2011","journal-title":"Appl Environ Microbiol"},{"issue":"4","key":"10.1016\/j.jcf.2023.03.007_bib0051","doi-asserted-by":"crossref","first-page":"e61176","DOI":"10.1371\/journal.pone.0061176","article-title":"Cystic fibrosis transmembrane conductance regulator (CFTR) allelic variants relate to shifts in faecal microbiota of cystic fibrosis patients","volume":"8","author":"Schippa","year":"2013","journal-title":"PLoS ONE"},{"issue":"3","key":"10.1016\/j.jcf.2023.03.007_bib0052","doi-asserted-by":"crossref","first-page":"396","DOI":"10.1093\/cid\/cit715","article-title":"Escherichia coli dysbiosis correlates with gastrointestinal dysfunction in children with cystic fibrosis","volume":"58","author":"Hoffman","year":"2014","journal-title":"Clin Infect Dis"},{"issue":"3","key":"10.1016\/j.jcf.2023.03.007_bib0053","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1136\/gutjnl-2014-306931","article-title":"The HLA-DQ2 genotype selects for early intestinal microbiota composition in infants at high risk of developing coeliac disease","volume":"64","author":"Olivares","year":"2015","journal-title":"Gut"},{"issue":"48","key":"10.1016\/j.jcf.2023.03.007_bib0054","doi-asserted-by":"crossref","first-page":"44641","DOI":"10.1074\/jbc.M105170200","article-title":"Butyrate suppression of colonocyte NF-kappa B activation and cellular proteasome activity","volume":"276","author":"Yin","year":"2001","journal-title":"J Biol Chem"},{"key":"10.1016\/j.jcf.2023.03.007_bib0055","doi-asserted-by":"crossref","first-page":"j5145","DOI":"10.1136\/bmj.j5145","article-title":"The role of the gut microbiome in systemic inflammatory disease","volume":"360","author":"Clemente","year":"2018","journal-title":"BMJ"},{"issue":"7480","key":"10.1016\/j.jcf.2023.03.007_bib0056","doi-asserted-by":"crossref","first-page":"451","DOI":"10.1038\/nature12726","article-title":"Metabolites produced by commensal bacteria promote peripheral regulatory T-cell generation","volume":"504","author":"Arpaia","year":"2013","journal-title":"Nature"},{"issue":"1","key":"10.1016\/j.jcf.2023.03.007_bib0057","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1186\/s12866-021-02305-z","article-title":"Infants with cystic fibrosis have altered fecal functional capacities with potential clinical and metabolic consequences","volume":"21","author":"Eng","year":"2021","journal-title":"BMC Microbiol"},{"issue":"1","key":"10.1016\/j.jcf.2023.03.007_bib0058","doi-asserted-by":"crossref","DOI":"10.1111\/aji.12778","article-title":"Breastfeeding and autoimmunity: programing health from the beginning","volume":"79","author":"Vieira Borba","year":"2018","journal-title":"Am J Reprod Immunol"},{"issue":"8","key":"10.1016\/j.jcf.2023.03.007_bib0059","doi-asserted-by":"crossref","first-page":"1228","DOI":"10.1111\/j.1651-2227.2007.00397.x","article-title":"Benefits of breastfeeding in cystic fibrosis: a single-centre follow-up survey","volume":"96","author":"Colombo","year":"2007","journal-title":"Acta Paediatr"},{"issue":"5","key":"10.1016\/j.jcf.2023.03.007_bib0060","doi-asserted-by":"crossref","DOI":"10.3390\/nu12051456","article-title":"Dietary habits and nutrition in rheumatoid arthritis: can diet influence disease development and clinical manifestations?","volume":"12","author":"Gioia","year":"2020","journal-title":"Nutrients"},{"issue":"11","key":"10.1016\/j.jcf.2023.03.007_bib0061","doi-asserted-by":"crossref","DOI":"10.1002\/14651858.CD002201.pub4","article-title":"Omega-3 fatty acids for cystic fibrosis","author":"Oliver","year":"2013","journal-title":"Cochrane Database Syst Rev"},{"issue":"5","key":"10.1016\/j.jcf.2023.03.007_bib0062","doi-asserted-by":"crossref","first-page":"832","DOI":"10.1016\/j.jada.2008.02.020","article-title":"Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review","volume":"108","author":"Stallings","year":"2008","journal-title":"J Am Diet Assoc"},{"issue":"5","key":"10.1016\/j.jcf.2023.03.007_bib0063","doi-asserted-by":"crossref","first-page":"777","DOI":"10.1016\/j.jcf.2022.03.010","article-title":"Obesity in Cystic fibrosis: prevalence, trends and associated factors data from the US cystic fibrosis foundation patient registry","volume":"21","author":"Szentpetery","year":"2022","journal-title":"J Cyst Fibros"},{"issue":"2","key":"10.1016\/j.jcf.2023.03.007_bib0064","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1016\/j.autrev.2012.07.007","article-title":"Can supplementation with vitamin D reduce the risk or modify the course of autoimmune diseases? A systematic review of the literature","volume":"12","author":"Antico","year":"2012","journal-title":"Autoimmun Rev"},{"issue":"2","key":"10.1016\/j.jcf.2023.03.007_bib0065","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1007\/s12016-012-8342-y","article-title":"Vitamin D in systemic and organ-specific autoimmune diseases","volume":"45","author":"Agmon-Levin","year":"2013","journal-title":"Clin Rev Allergy Immunol"},{"issue":"4","key":"10.1016\/j.jcf.2023.03.007_bib0066","first-page":"619","article-title":"Prevalence of inflammatory bowel disease in pediatric and adult populations: recent estimates from large national databases in the United States, 2007-2016","volume":"26","author":"Ye","year":"2020","journal-title":"Inflamm Bowel Dis"},{"issue":"4","key":"10.1016\/j.jcf.2023.03.007_bib0067","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1002\/ppul.22967","article-title":"The cystic fibrosis gender gap: potential roles of estrogen","volume":"49","author":"Sweezey","year":"2014","journal-title":"Pediatr Pulmonol"},{"issue":"8","key":"10.1016\/j.jcf.2023.03.007_bib0068","doi-asserted-by":"crossref","first-page":"1338","DOI":"10.2337\/dc16-0020","article-title":"Does beta-cell autoimmunity play a role in cystic fibrosis-related diabetes? Analysis based on the German\/Austrian diabetes Patienten Verlaufsdokumentation registry","volume":"39","author":"Konrad","year":"2016","journal-title":"Diabetes Care"},{"issue":"2","key":"10.1016\/j.jcf.2023.03.007_bib0069","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1080\/08916934.2021.2021193","article-title":"Serum anti-PAD4 autoantibodies are present in cystic fibrosis children and increase with age and lung disease severity","volume":"55","author":"Linnemann","year":"2022","journal-title":"Autoimmunity"},{"issue":"5","key":"10.1016\/j.jcf.2023.03.007_bib0070","doi-asserted-by":"crossref","first-page":"1079","DOI":"10.1183\/09031936.00006009","article-title":"Prevalence and clinical significance of auto-antibodies in adults with cystic fibrosis","volume":"34","author":"Lachenal","year":"2009","journal-title":"Eur Respir J"},{"issue":"6","key":"10.1016\/j.jcf.2023.03.007_bib0071","doi-asserted-by":"crossref","first-page":"823","DOI":"10.1016\/j.cgh.2017.06.037","article-title":"Global prevalence of celiac disease: systematic review and meta-analysis","volume":"16","author":"Singh","year":"2018","journal-title":"Clin Gastroenterol Hepatol"},{"issue":"6","key":"10.1016\/j.jcf.2023.03.007_bib0072","doi-asserted-by":"crossref","first-page":"512","DOI":"10.1136\/adc.2003.029603","article-title":"Mass screening for coeliac disease using antihuman transglutaminase antibody assay","volume":"89","author":"Tommasini","year":"2004","journal-title":"Arch Dis Child"},{"issue":"3","key":"10.1016\/j.jcf.2023.03.007_bib0073","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1001\/archinte.163.3.286","article-title":"Prevalence of celiac disease in at-risk and not-at-risk groups in the United States: a large multicenter study","volume":"163","author":"Fasano","year":"2003","journal-title":"Arch Intern Med"},{"issue":"7","key":"10.1016\/j.jcf.2023.03.007_bib0074","doi-asserted-by":"crossref","first-page":"960","DOI":"10.1136\/gut.52.7.960","article-title":"Seroprevalence, correlates, and characteristics of undetected coeliac disease in England","volume":"52","author":"West","year":"2003","journal-title":"Gut"},{"issue":"9","key":"10.1016\/j.jcf.2023.03.007_bib0075","doi-asserted-by":"crossref","DOI":"10.3390\/jpm11090859","article-title":"Increased prevalence of celiac disease in patients with cystic fibrosis: a systematic review and meta-analysis","volume":"11","author":"Imrei","year":"2021","journal-title":"J Pers Med"},{"issue":"1","key":"10.1016\/j.jcf.2023.03.007_bib0076","first-page":"1055","article-title":"Cystic fibrosis and celiac disease: mere coincidence?","volume":"4","author":"Broekaert","year":"2016","journal-title":"JSM Gastroenterol Hepatol"},{"issue":"1","key":"10.1016\/j.jcf.2023.03.007_bib0077","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1007\/s13317-016-0073-2","article-title":"Simultaneous detection of celiac disease-specific IgA antibodies and total IgA","volume":"7","author":"Grossmann","year":"2016","journal-title":"Auto Immun Highlights"},{"issue":"1","key":"10.1016\/j.jcf.2023.03.007_bib0078","doi-asserted-by":"crossref","first-page":"115","DOI":"10.18388\/abp.2010_2382","article-title":"Cystic fibrosis is a risk factor for celiac disease","volume":"57","author":"Walkowiak","year":"2010","journal-title":"Acta Biochim Pol"},{"key":"10.1016\/j.jcf.2023.03.007_bib0079","first-page":"418","article-title":"Cystic fibrosis and celiac disease: TTG screening results indicate a common association","volume":"45","author":"Davidson","year":"2010","journal-title":"Pediatr Pulmonol"},{"key":"10.1016\/j.jcf.2023.03.007_bib0080","first-page":"9","article-title":"Abdominal pain in cystic fibrosis","volume":"88","author":"Littlewood","year":"1995","journal-title":"J Royal Soc Med"},{"issue":"5","key":"10.1016\/j.jcf.2023.03.007_bib0081","doi-asserted-by":"crossref","first-page":"784","DOI":"10.1111\/j.1651-2227.1989.tb11146.x","article-title":"Incidence of celiac disease in a cystic fibrosis population","volume":"78","author":"Valleta","year":"1989","journal-title":"Acta Paediatr Scand"},{"key":"10.1016\/j.jcf.2023.03.007_bib0082","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1007\/s12456-012-0020-8","article-title":"Zijn we tevreden met e\u00b4e\u00b4n diagnose?","volume":"80","author":"Ras","year":"2012","journal-title":"Tijdschr Kindergeneeskd"},{"key":"10.1016\/j.jcf.2023.03.007_bib0083","article-title":"Cistic fibroses in children and adolescents in Fortaleza-CE: a epidemiology study and comorbidities","volume":"51","author":"De Castro ESC","year":"2016","journal-title":"Pediatric Pulmonol"},{"issue":"1","key":"10.1016\/j.jcf.2023.03.007_bib0084","doi-asserted-by":"crossref","first-page":"106","DOI":"10.1016\/S1744-1161(11)70271-X","article-title":"Nutritional status and treatment in children with Cystic Fibrosis","volume":"6","author":"Anton","year":"2011","journal-title":"Clin Nutr Supplements"},{"key":"10.1016\/j.jcf.2023.03.007_bib0085","doi-asserted-by":"crossref","DOI":"10.1016\/S1569-1993(17)30639-2","article-title":"Association between cystic fibrosis and celiac disease, how much is that frequent?","volume":"16","author":"Masip","year":"2017","journal-title":"J Cyst Fibros"},{"key":"10.1016\/j.jcf.2023.03.007_bib0086","doi-asserted-by":"crossref","first-page":"39","DOI":"10.5152\/EurJTher.2018.701","article-title":"The frequency of celiac disease in Turkish children with cystic fibrosis","volume":"25","author":"Sahin","year":"2019","journal-title":"Eur J Ther"},{"issue":"3","key":"10.1016\/j.jcf.2023.03.007_bib0087","doi-asserted-by":"crossref","first-page":"198","DOI":"10.1016\/j.jcf.2009.02.002","article-title":"Co-morbidity of cystic fibrosis and celiac disease in Scandinavian cystic fibrosis patients","volume":"8","author":"Fluge","year":"2009","journal-title":"J Cyst Fibros"},{"issue":"1","key":"10.1016\/j.jcf.2023.03.007_bib0088","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1186\/s12916-019-1380-z","article-title":"Celiac disease: a comprehensive current review","volume":"17","author":"Caio","year":"2019","journal-title":"BMC Med"},{"issue":"3","key":"10.1016\/j.jcf.2023.03.007_bib0089","first-page":"260","article-title":"Effects of therapy with lansoprazole on intestinal permeability and inflammation in young cystic fibrosis patients","volume":"33","author":"Hendriks","year":"2001","journal-title":"J Pediatr Gastroenterol Nutr"},{"issue":"43","key":"10.1016\/j.jcf.2023.03.007_bib0090","doi-asserted-by":"crossref","first-page":"4492","DOI":"10.18632\/oncotarget.27037","article-title":"Cystic fibrosis transmembrane conductance regulator (CFTR) and autophagy: hereditary defects in cystic fibrosis versus gluten-mediated inhibition in celiac disease","volume":"10","author":"Maiuri","year":"2019","journal-title":"Oncotarget"},{"issue":"2","key":"10.1016\/j.jcf.2023.03.007_bib0091","doi-asserted-by":"crossref","DOI":"10.15252\/embj.2018100101","article-title":"A pathogenic role for cystic fibrosis transmembrane conductance regulator in celiac disease","volume":"38","author":"Villella","year":"2019","journal-title":"EMBO J"},{"issue":"4","key":"10.1016\/j.jcf.2023.03.007_bib0092","doi-asserted-by":"crossref","first-page":"880","DOI":"10.1007\/BF02087437","article-title":"Crohn's disease and cystic fibrosis","volume":"39","author":"Lloyd-Still","year":"1994","journal-title":"Dig Dis Sci"},{"issue":"4","key":"10.1016\/j.jcf.2023.03.007_bib0093","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1016\/j.jcf.2010.03.010","article-title":"Cystic fibrosis gene mutations and gastrointestinal diseases","volume":"9","author":"Bahmanyar","year":"2010","journal-title":"J Cyst Fibros"},{"key":"10.1016\/j.jcf.2023.03.007_bib0094","first-page":"156","article-title":"Inflammatory bowel disease in cystic fibrosis: is it more prevalent?","author":"Kesler","year":"2019","journal-title":"Gastroenterology"},{"issue":"89","key":"10.1016\/j.jcf.2023.03.007_bib0095","first-page":"162","article-title":"Clinical utility of serological markers in inflammatory bowel disease","volume":"56","author":"Mokrowiecka","year":"2009","journal-title":"Hepatogastroenterology"},{"key":"10.1016\/j.jcf.2023.03.007_bib0096","doi-asserted-by":"crossref","first-page":"89","DOI":"10.3389\/fmicb.2015.00089","article-title":"The lung mycobiome: an emerging field of the human respiratory microbiome","volume":"6","author":"Nguyen","year":"2015","journal-title":"Front Microbiol"},{"issue":"5","key":"10.1016\/j.jcf.2023.03.007_bib0097","doi-asserted-by":"crossref","first-page":"322","DOI":"10.3109\/00365513.2010.486869","article-title":"Small intestine bacterial overgrowth does not correspond to intestinal inflammation in cystic fibrosis","volume":"70","author":"Lisowska","year":"2010","journal-title":"Scand J Clin Lab Invest"},{"key":"10.1016\/j.jcf.2023.03.007_bib0098","doi-asserted-by":"crossref","DOI":"10.3389\/fped.2020.585275","article-title":"Vasculitis in Cystic Fibrosis","volume":"8","author":"Sposito","year":"2020","journal-title":"Front Pediatr"},{"issue":"4","key":"10.1016\/j.jcf.2023.03.007_bib0099","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1016\/j.clindermatol.2018.04.012","article-title":"Cutaneous vasculitis in rheumatologic disease: current concepts of skin and systemic manifestations","volume":"36","author":"Micheletti","year":"2018","journal-title":"Clin Dermatol"},{"issue":"Suppl 19","key":"10.1016\/j.jcf.2023.03.007_bib0100","first-page":"38","article-title":"Vasculitis and arthropathy in cystic fibrosis","volume":"85","author":"Hodson","year":"1992","journal-title":"J R Soc Med"},{"issue":"6","key":"10.1016\/j.jcf.2023.03.007_bib0101","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1016\/j.jcf.2010.08.017","article-title":"Treatment of cystic fibrosis associated cutaneous vasculitis with chloroquine","volume":"9","author":"Molyneux","year":"2010","journal-title":"J Cyst Fibros"},{"issue":"2","key":"10.1016\/j.jcf.2023.03.007_bib0102","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1016\/j.jbspin.2013.09.003","article-title":"Prevalence and incidence of juvenile idiopathic arthritis: a systematic review","volume":"81","author":"Thierry","year":"2014","journal-title":"Joint Bone Spine"},{"issue":"9","key":"10.1016\/j.jcf.2023.03.007_bib0103","doi-asserted-by":"crossref","first-page":"1551","DOI":"10.1007\/s00296-017-3726-1","article-title":"Prevalence of rheumatoid arthritis in the United States adult population in healthcare claims databases, 2004-2014","volume":"37","author":"Hunter","year":"2017","journal-title":"Rheumatol Int"},{"issue":"3","key":"10.1016\/j.jcf.2023.03.007_bib0104","first-page":"319","article-title":"Seropositive rheumatoid arthritis in a patient with cystic fibrosis","volume":"134","author":"Sagransky","year":"1980","journal-title":"Am J Dis Child"},{"issue":"9","key":"10.1016\/j.jcf.2023.03.007_bib0105","doi-asserted-by":"crossref","DOI":"10.1136\/bcr-2020-234305","article-title":"Rheumatoid arthritis in a patient with cystic fibrosis: challenging treatment options","volume":"13","author":"Delaney-Nelson","year":"2020","journal-title":"BMJ Case Rep"},{"issue":"2","key":"10.1016\/j.jcf.2023.03.007_bib0106","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1093\/rheumatology\/28.2.179","article-title":"Cystic fibrosis and rheumatoid arthritis","volume":"28","author":"Gardiner","year":"1989","journal-title":"Br J Rheumatol"},{"issue":"1","key":"10.1016\/j.jcf.2023.03.007_bib0107","article-title":"Anti-inflammatory drugs and analgesics for managing symptoms in people with cystic fibrosis-related arthritis","volume":"2016","author":"Thornton","year":"2016","journal-title":"Cochrane Database Syst Rev"},{"issue":"4","key":"10.1016\/j.jcf.2023.03.007_bib0108","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1136\/adc.59.4.377","article-title":"Arthritis in cystic fibrosis","volume":"59","author":"Schidlow","year":"1984","journal-title":"Arch Dis Child"},{"issue":"4","key":"10.1016\/j.jcf.2023.03.007_bib0109","doi-asserted-by":"crossref","first-page":"594","DOI":"10.1016\/S0022-3476(79)80018-9","article-title":"Episodic arthritis in children with cystic fibrosis","volume":"94","author":"Newman","year":"1979","journal-title":"J Pediatr"},{"issue":"1","key":"10.1016\/j.jcf.2023.03.007_bib0110","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/0007-0971(78)90007-4","article-title":"Pulmonary infection and rheumatoid arthritis","volume":"72","author":"Mathieu","year":"1978","journal-title":"Br J Dis Chest"},{"issue":"3","key":"10.1016\/j.jcf.2023.03.007_bib0111","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1136\/ard.47.3.218","article-title":"The arthropathy of cystic fibrosis","volume":"47","author":"Dixey","year":"1988","journal-title":"Ann Rheum Dis"},{"issue":"3","key":"10.1016\/j.jcf.2023.03.007_bib0112","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1097\/01.mpg.0000178439.64675.8d","article-title":"Gastrointestinal outcomes and confounders in cystic fibrosis","volume":"41","author":"Borowitz","year":"2005","journal-title":"J Pediatr Gastroenterol Nutr"},{"issue":"6","key":"10.1016\/j.jcf.2023.03.007_bib0113","doi-asserted-by":"crossref","first-page":"e87","DOI":"10.1016\/j.jcf.2021.05.003","article-title":"Risk factors for cystic fibrosis arthropathy: data from the German cystic fibrosis registry","volume":"20","author":"Grehn","year":"2021","journal-title":"J Cyst Fibros"},{"issue":"3","key":"10.1016\/j.jcf.2023.03.007_bib0114","doi-asserted-by":"crossref","first-page":"374","DOI":"10.1016\/S0022-3476(05)83279-2","article-title":"Prevalence of circulating immune complexes in patients with cystic fibrosis and arthritis","volume":"125","author":"Wulffraat","year":"1994","journal-title":"J Pediatr"},{"issue":"2","key":"10.1016\/j.jcf.2023.03.007_bib0115","doi-asserted-by":"crossref","first-page":"212","DOI":"10.1513\/AnnalsATS.201907-493OC","article-title":"Changes in airway microbiome and inflammation with Ivacaftor treatment in patients with cystic fibrosis and the G551D mutation","volume":"17","author":"Harris","year":"2020","journal-title":"Ann Am Thorac Soc."},{"issue":"12","key":"10.1016\/j.jcf.2023.03.007_bib0116","doi-asserted-by":"crossref","first-page":"1617","DOI":"10.1164\/rccm.201609-1954OC","article-title":"Restoring cystic fibrosis transmembrane conductance regulator function reduces airway bacteria and inflammation in people with cystic fibrosis and chronic lung infections","volume":"195","author":"Hisert","year":"2017","journal-title":"Am J Respir Crit Care Med"},{"issue":"17","key":"10.1016\/j.jcf.2023.03.007_bib0117","doi-asserted-by":"crossref","DOI":"10.3390\/ijms21176379","article-title":"Airway inflammation and host responses in the era of CFTR modulators","volume":"21","author":"Keown","year":"2020","journal-title":"Int J Mol Sci"},{"issue":"1","key":"10.1016\/j.jcf.2023.03.007_bib0118","doi-asserted-by":"crossref","first-page":"17834","DOI":"10.1038\/s41598-018-36364-6","article-title":"Impact of CFTR modulation with Ivacaftor on gut microbiota and intestinal inflammation","volume":"8","author":"Ooi","year":"2018","journal-title":"Sci Rep"},{"issue":"7","key":"10.1016\/j.jcf.2023.03.007_bib0119","doi-asserted-by":"crossref","first-page":"999","DOI":"10.1182\/blood-2014-02-555268","article-title":"A neutrophil intrinsic impairment affecting Rab27a and degranulation in cystic fibrosis is corrected by CFTR potentiator therapy","volume":"124","author":"Pohl","year":"2014","journal-title":"Blood"},{"issue":"3","key":"10.1016\/j.jcf.2023.03.007_bib0120","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0213216","article-title":"Clinical validation of an evidence-based method to adjust Pancreatic Enzyme Replacement Therapy through a prospective interventional study in paediatric patients with Cystic Fibrosis","volume":"14","author":"Calvo-Lerma","year":"2019","journal-title":"PLoS ONE"},{"issue":"Suppl 1","key":"10.1016\/j.jcf.2023.03.007_bib0121","doi-asserted-by":"crossref","first-page":"S1","DOI":"10.1086\/676882","article-title":"Infection prevention and control guideline for cystic fibrosis: 2013 update","volume":"35","author":"Saiman","year":"2014","journal-title":"Infect Control Hosp Epidemiol"},{"issue":"3","key":"10.1016\/j.jcf.2023.03.007_bib0122","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1513\/AnnalsATS.201908-638VP","article-title":"Creating a virtual environment to enable two-way learning about the relevance of clinical research","volume":"17","author":"Borowitz","year":"2020","journal-title":"Ann Am Thorac Soc"}],"container-title":["Journal of Cystic Fibrosis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1569199323000711?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1569199323000711?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T21:09:27Z","timestamp":1762031367000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1569199323000711"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,11]]},"references-count":122,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2023,11]]}},"alternative-id":["S1569199323000711"],"URL":"https:\/\/doi.org\/10.1016\/j.jcf.2023.03.007","relation":{},"ISSN":["1569-1993"],"issn-type":[{"type":"print","value":"1569-1993"}],"subject":[],"published":{"date-parts":[[2023,11]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Autoimmunity in people with cystic fibrosis","name":"articletitle","label":"Article Title"},{"value":"Journal of Cystic Fibrosis","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jcf.2023.03.007","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2023 European Cystic Fibrosis Society. Published by Elsevier B.V.","name":"copyright","label":"Copyright"}]}}