{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T21:28:09Z","timestamp":1790803689032,"version":"4.1.0"},"reference-count":38,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2020,2,10]],"date-time":"2020-02-10T00:00:00Z","timestamp":1581292800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"EU grant FP7 Health","award":["305653-chILD-EU"],"award-info":[{"award-number":["305653-chILD-EU"]}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2020,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objectives<\/jats:title><jats:p>Pediatric lymphocytic interstitial pneumonia (LIP) and follicular bronchiolitis (FB) are poorly characterized lymphoproliferative disorders. We present and quantify demographics, radiological and histopathologic patterns, treatments and their responses, and outcomes in non\u2010HIV\u2010infected children with LIP and FB.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This structured registry\u2010based study included a retrospective chart review, blinded analysis of imaging studies and lung biopsies, genetic testing, and evaluation of treatments and outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of the 13 patients (eight females) studied, eight had FB, four had combined LIP\/FB, and one had isolated LIP; diagnoses were highly concordant between the pathologists. Most patients became symptomatic during the first 2 years of life, with a mean lag time to diagnosis of 4 years. The most common symptoms were coughing and respiratory infections (11 out of 13 each), dyspnea (10 out of 13), and wheezing (eight out of 13). Autoantibodies were found in eight out of 13 patients. In three patients, disease\u2010causing mutations in the <jats:italic>COPA<\/jats:italic> gene were identified. CT revealed hilar lymphadenopathy (five out of 12), ground\u2010glass opacity (eight out of 12), consolidation (five out of 12), and cysts (four out of 13). Systemic steroids as intravenous pulses (11 out of 13) or oral intake (10 out of 13) were the main treatments and showed high response rates of 100% and 90%, respectively. Within the mean observation period of 68 months, all children had chronic courses, eight out of 13 had\u00a0severe diseases, two died, and one worsened.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Children with LIP\/FB have chronic diseases that occurred in early childhood and were commonly associated with immune dysregulation as well as high morbidity and mortality. Early diagnosis and treatment may be crucial to improve the outcome.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.24680","type":"journal-article","created":{"date-parts":[[2020,2,10]],"date-time":"2020-02-10T18:50:42Z","timestamp":1581360642000},"page":"909-917","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["Lymphocytic interstitial pneumonia and follicular bronchiolitis in children: A registry\u2010based case series"],"prefix":"10.1002","volume":"55","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2838-593X","authenticated-orcid":false,"given":"Freerk","family":"Prenzel","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Center for Pediatric Research Leipzig (CPL) University of Leipzig Medical Center  Leipzig Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacqueline","family":"Harfst","sequence":"additional","affiliation":[{"name":"Hauner Children's Hospital and KUBUS Research Center University of Munich  Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolaus","family":"Schwerk","sequence":"additional","affiliation":[{"name":"Clinic for Pediatric Pneumology, Allergology, and Neonatology Hannover Medical School  Hannover Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frank","family":"Ahrens","sequence":"additional","affiliation":[{"name":"Altonaer Children's Hospital  Hamburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ernst","family":"Rietschel","sequence":"additional","affiliation":[{"name":"University Children's Hospital University of Cologne  Cologne Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabina","family":"Schmitt\u2010Groh\u00e9","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University Hospital Bonn  Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sune M. 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