Universität zu Köln

Prenzel, Freerk ORCID: 0000-0002-2838-593X, Harfst, Jacqueline, Schwerk, Nicolaus, Ahrens, Frank, Rietschel, Ernst, Schmitt-Groh, Sabina, Rubak, Sune M. L., Poplawska, Krystyna, Baden, Winfried, Vogel, Mandy, Hollizeck, Sebastian, Ley-Zaporozhan, Julia, Brasch, Frank, Reu, Simone and Griese, Matthias (2020). Lymphocytic interstitial pneumonia and follicular bronchiolitis in children: A registry-based case series. Pediatr. Pulmonol., 55 (4). S. 909 - 918. HOBOKEN: WILEY. ISSN 1099-0496

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Abstract

Objectives: 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-HIV-infected children with LIP and FB. Methods: This structured registry-based study included a retrospective chart review, blinded analysis of imaging studies and lung biopsies, genetic testing, and evaluation of treatments and outcomes. Results: 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-causing mutations in the COPA gene were identified. CT revealed hilar lymphadenopathy (five out of 12), ground-glass 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 severe diseases, two died, and one worsened. Conclusions: 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.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Prenzel, Freerk UNSPECIFIED orcid.org/0000-0002-2838-593X UNSPECIFIED Harfst, Jacqueline UNSPECIFIED UNSPECIFIED UNSPECIFIED Schwerk, Nicolaus UNSPECIFIED UNSPECIFIED UNSPECIFIED Ahrens, Frank UNSPECIFIED UNSPECIFIED UNSPECIFIED Rietschel, Ernst UNSPECIFIED UNSPECIFIED UNSPECIFIED Schmitt-Groh, Sabina UNSPECIFIED UNSPECIFIED UNSPECIFIED Rubak, Sune M. L. UNSPECIFIED UNSPECIFIED UNSPECIFIED Poplawska, Krystyna UNSPECIFIED UNSPECIFIED UNSPECIFIED Baden, Winfried UNSPECIFIED UNSPECIFIED UNSPECIFIED Vogel, Mandy UNSPECIFIED UNSPECIFIED UNSPECIFIED Hollizeck, Sebastian UNSPECIFIED UNSPECIFIED UNSPECIFIED Ley-Zaporozhan, Julia UNSPECIFIED UNSPECIFIED UNSPECIFIED Brasch, Frank UNSPECIFIED UNSPECIFIED UNSPECIFIED Reu, Simone UNSPECIFIED UNSPECIFIED UNSPECIFIED Griese, Matthias UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-345185
DOI:	10.1002/ppul.24680
Journal or Publication Title:	Pediatr. Pulmonol.
Volume:	55
Number:	4
Page Range:	S. 909 - 918
Date:	2020
Publisher:	WILEY
Place of Publication:	HOBOKEN
ISSN:	1099-0496
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language DIFFUSE LUNG-DISEASE; IMMUNE DYSREGULATION; LYMPHOPROLIFERATIVE DISORDERS; CLINICAL-PRACTICE; CLASSIFICATION; BRONCHITIS; CHILDHOOD; MUTATIONS; AGREEMENT Multiple languages Pediatrics; Respiratory System Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/34518