Lymphocytic interstitial pneumonia and follicular bronchiolitis in children: A registry-based case series.
Adolescent
Age of Onset
Biopsy
Bronchitis
/ complications
Child
Child, Preschool
Chronic Disease
Cough
/ etiology
Diagnosis, Differential
Dyspnea
/ etiology
Female
Genetic Testing
Humans
Infant
Lung
/ diagnostic imaging
Lung Diseases
/ diagnosis
Lung Diseases, Interstitial
/ complications
Male
Registries
Respiratory Sounds
/ etiology
Respiratory Tract Infections
/ etiology
Retrospective Studies
Tomography, X-Ray Computed
COPA syndrome
immune dysregulatory disease
interstitial lung disease in children
rare pediatric lung disease
Journal
Pediatric pulmonology
ISSN: 1099-0496
Titre abrégé: Pediatr Pulmonol
Pays: United States
ID NLM: 8510590
Informations de publication
Date de publication:
04 2020
04 2020
Historique:
received:
19
07
2019
accepted:
26
01
2020
pubmed:
11
2
2020
medline:
11
11
2020
entrez:
11
2
2020
Statut:
ppublish
Résumé
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. 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. 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. 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.
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
909-917Informations de copyright
© 2020 Wiley Periodicals, Inc.
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