成年期的儿童间质性肺病幸存者：一项欧洲合作研究。

Childhood interstitial lung disease survivors in adulthood: a European collaborative study.

作者信息

Manali Effrosyni D, Griese Matthias, Nathan Nadia, Uzunhan Yurdagül, Borie Raphael, Michel Katarzyna, Schwerk Nicolaus, Fijolek Justyna, Radzikowska Elżbieta, Chua Felix, Pabary Rishi, Mogulkoc Nesrin, McCarthy Cormac, Kallieri Maria, Papaioannou Andriana I, Kiper Nural, Koziar Vasakova Martina, Lacina Ladislav, Molina-Molina Maria, Torrent-Vernetta Alba, Tsiligiannis Theofanis, Karadag Bulent, Kokosi Maria, Renzoni Elisabetta A, van Moorsel Coline H M, Campo Ilaria, Bendstrup Elisabeth, Prior Thomas Skovhus, Prasse Antje, Bonella Francesco, Cottin Vincent, Diesler Rémi, Froidure Antoine, Kolilekas Lykourgos, Fotis Lampros, Douros Konstantinos, Kaditis Athanasios G, Jeny Florence, Chauveau Simon, Nunes Hilario, Dahbia Azrine, Mariani Francesca, van der Vis Joanne J, Groen Karlijn, Erdem Eralp Ela, Gokdemir Yasemin, Kocakaya Derya, Olgun Yildizeli Sehnaz, Yalçın Ebru, Emiralioğlu Nagehan, Nayir Buyuksahin Halime, O'Brien Helen, Karcıoglu Oguz, Can Demet, Ezircan Alper, Kartal Ozturk Gokcen, Ocal Nesrin, Yuksel Hasan, Narin Tongal Sedef, Safrankova Martina, Kourtesi Katerina, Louvrier Camille, Kannengiesser Caroline, Fabre Aurelie, Legendre Marie, Crestani Bruno, Pohunek Petr, Bush Andrew, Papiris Spyros A

机构信息

2nd Pulmonary Medicine Department, General University Hospital "Attikon", Medical School, National and Kapodistrian University of Athens, Athens, Greece

Department of Pediatric Pneumology, Dr von Hauner Children's Hospital, Ludwig-Maximilians-University, German Center for Lung Research, Munich, Germany.

出版信息

Eur Respir J. 2025 Feb 13;65(2). doi: 10.1183/13993003.00680-2024. Print 2025 Feb.

DOI:10.1183/13993003.00680-2024

PMID:39638417

Abstract

BACKGROUND

Interstitial lung disease is rarer in children than adults, but, with increasing diagnostic awareness, more cases are being discovered. The prognosis of childhood interstitial lung disease is often poor, but increasing numbers are now surviving into adulthood.

AIM

To characterise childhood interstitial lung disease survivors and identify their impact on adult interstitial lung disease centres.

METHODS

This was a European study (34 adult and childhood interstitial lung disease centres) reporting incident/prevalent cases of childhood interstitial lung disease survivors from January to July 2023. Epidemiological, clinical, physiological and genetic data were collected.

RESULTS

244 patients were identified with a median (interquartile range) age at diagnosis of 12.5 years (6-16 years) and age at study inclusion of 25 years (22-33 years), with 51% male, 86% nonsmokers and a median (interquartile range) % predicted forced vital capacity of 70% (47-89%) and diffusing capacity of the lungs for carbon monoxide of 48% (32-75%). 32% were prescribed long-term oxygen and 227 (93%) were followed up in adult centres whereas 17 (7%) never transitioned. The commonest diagnoses (82%) were childhood interstitial lung disease category B1 (sarcoidosis, hemosiderosis, connective tissue disorders, vasculitis) at 35%, A4 (surfactant-related) at 21%, B2 (bronchiolitis obliterans, hypersensitivity pneumonitis) at 14% and Bz (unclassified interstitial lung disease) at 13%. Bz patients had the worst functional status. 60% of all patients were still being prescribed corticosteroids. Re-specification of diagnosis and treatment were made after transition for 9.8% and 16% of patients, respectively. Not all childhood interstitial lung disease diagnoses were recognised in adult interstitial lung disease classifications.

CONCLUSION

Childhood interstitial lung disease survivors are seen in most adult interstitial lung disease centres and only a minority continue follow-up in paediatric centres. Survivors have a significant loss of lung function. The heterogeneity of their aetiologies and therapeutic requirements has a real impact on adult interstitial lung disease centres. Re-specification of diagnosis and treatment may contribute to precision and personalisation of management.

摘要

背景

间质性肺疾病在儿童中比成人中更为罕见，但随着诊断意识的提高，发现的病例越来越多。儿童间质性肺疾病的预后通常较差，但现在存活至成年的人数在增加。

目的

描述儿童间质性肺疾病幸存者的特征，并确定他们对成人间质性肺疾病中心的影响。

方法

这是一项欧洲研究（34个成人和儿童间质性肺疾病中心），报告了2023年1月至7月儿童间质性肺疾病幸存者的新发病例/现患病例。收集了流行病学、临床、生理和遗传数据。

结果

共识别出244例患者，诊断时的中位（四分位间距）年龄为12.5岁（6 - 16岁），纳入研究时的年龄为25岁（22 - 33岁），其中51%为男性，86%为非吸烟者，预测的用力肺活量中位数（四分位间距）为70%（47 - 89%），肺一氧化碳弥散量为48%（32 - 75%）。32%的患者被处方长期吸氧，227例（93%）在成人中心接受随访，而17例（7%）从未转诊。最常见的诊断（82%）为儿童间质性肺疾病B1类（结节病、含铁血黄素沉着症、结缔组织病、血管炎），占35%；A4类（与表面活性剂相关），占21%；B2类（闭塞性细支气管炎、过敏性肺炎），占14%；Bz类（未分类的间质性肺疾病），占13%。Bz类患者的功能状态最差。所有患者中有60%仍在使用糖皮质激素。转诊后分别有9.8%和16%的患者重新进行了诊断和治疗。并非所有儿童间质性肺疾病诊断在成人间质性肺疾病分类中都能得到认可。