Pulmonary Fibrosis – From Childhood to Adulthood

Interstitial lung diseases (ILD) in childhood represent a heterogeneous group of rare lung diseases with high morbidity and mortality. Many children are affected during their first years of life. However, there is a lack of valid data on their long-term progression into adulthood. An international team of researchers, including members of the German Center for Lung Research (DZL), has now addressed this topic, focusing on individuals who have reached adulthood with an ILD. The findings were recently published in two papers.

Childhood interstitial lung disease (chILD) is an umbrella term for a heterogeneous group of more than 200 rare diffuse lung diseases that manifest during childhood and vary significantly in their causes and progression. While many children succumb to the disease within their first year of life, others survive into adulthood. A significant proportion of those affected develop pulmonary fibrosis over the course of the disease. The number of patients who reach adulthood with chILD is steadily increasing. How do these individuals cope with their condition, and what is the process of transitioning from pediatric care to adult healthcare? Researchers from the DZL, including Prof. Dr. Matthias Griese (Munich) and PD Dr. Nicolaus Schwerk (Hannover), have collaborated with an international team to explore these questions.

Over the past decade, the spectrum of specific chILD entities has expanded significantly due to advancements in genetic testing, leading to the discovery of new diseases. However, much of the knowledge in this field is based on small case series published across various disciplines in clinical and molecular journals. In particular, the prevalence, management, and progression of pulmonary fibrosis in children with chILD remain poorly characterized. "There is a significant need for further research in this area," says PD Dr. Schwerk from the Department of Pediatric Pulmonology, Allergology, and Neonatology at Hannover Medical School (MHH). "While we have clear guidelines for diagnosing and treating interstitial lung diseases in adults, pediatric guidelines exist but do not address the specific disease entities and their therapies due to the lack of data from controlled studies."

In their review, the team identified 47 different forms of chILD from registry data that can potentially lead to pulmonary fibrosis. Many of these diseases are genetically determined and may manifest differently as the child grows. The researchers emphasize that children with chILD who develop pulmonary fibrosis often face progressive lung damage into adulthood. The study authors call for intensified research and a deeper understanding of chILD to prevent disease progression through targeted treatment approaches, improve care for affected children, and ensure a seamless transition to adult care. These findings highlight the importance of early diagnosis and specialized care for children with interstitial lung diseases to minimize long-term health consequences.

As coordinator of the European Network for Rare Lung Diseases (chILD-EU), Prof. Griese aimed to take this effort further. In a study published in the European Respiratory Journal, he, along with Nicolaus Schwerk and other European researchers, investigated for the first time how former chILD patients transition into adult patients with pulmonary fibrosis. Their findings indicate that when patients transition from pediatric to specialized adult care, their diagnoses often, but not always, align with ILD categories used in adult classifications. As a result, patients may be misclassified into unsuitable diagnostic categories. According to the researchers, the implications of this non-standardized transition to adult ILD care require further investigation. Revising and refining diagnoses and treatments could enhance the precision and personalization of patient care. Finally, updated, evidence-based guidelines for diagnosing and treating interstitial lung diseases in children and ensuring a smooth transition to adult care are urgently needed.

The original publications can be found here:

Pulmonary fibrosis may begin in infancy: from childhood to adult interstitial lung disease

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

 

Text: DZL & BREATH

Photo: MHH/ Tom Figil

BREATH Scientist and Senior Physician at the Department of Pediatric Pulmonology, Allergology, and Neonatology at Hannover Medical School (MHH)