Childhood interstitial lung disease
Childhood interstitial lung disease (chILD) is a broad collection of diverse interstitial lung diseases (ILDs) with an age of onset ranging from 0 to 18 years1-3
PREVALENCE OF chILD
Prevalence of chILD in Europe is estimated to be between 1.3 and 46.5 cases per million children*4–6
SIGNS, SYMPTOMS AND COMORBIDITIES OF chILD
The severity of chILD presentation is highly variable, ranging from mild nonspecific symptoms to very severe disease7
Despite the heterogeneity within chILD, there is significant overlap in its clinical manifestations7
The most common signs and symptoms of chILD include:7–27
Comorbidities are seen in almost half of all patients with chILD (48.1%), and 27.4% report ≥1 comorbidity,17 with the most frequent comorbidities being:15–17,20
Nervous system disease
Diseases of musculoskeletal system (6.6%–9.0%)
Skin disease (4.8%–8.0%)
Pulmonary fibrosis in chILD
The proportion of patients with chILD classified as having pulmonary fibrosis ranges from 2% to 26%27–29
- In a UK study, pulmonary fibrosis was reported using biopsy in 2% (n=2/93) of patients with chILD aged <2 years and in 7% (n=8/118) aged 2–18 years28
- In a French study, pulmonary fibrosis was reported using HRCT and histology in 4% (n=4/119) of patients with chILD29
- In a European study, pulmonary fibrosis was reported mostly using lung biopsy in 26% (n=46/177) of patients with chILD27
In ILDs in general, HRCT is essential for reaching an accurate diagnosis of pulmonary fibrosis30
Disease severity and progression
Disease severity in chILD can be assessed using the Fan severity score by establishing the patient history and undertaking a physical examination, whose increasing value is associated with significantly poorer outcomes, including decreased survival.24,31 Higher Fan severity scores have also been reported following ≥1 incident(s) of acute exacerbation of ILD (AE-ILD)10
Although there is no single validated threshold for clinically significant ILD progression in chILD, the clinical course of chILD can also be assessed using the Fan severity score, as well as monitoring the child’s nutritional status by observing their calorie count and growth parameters32
MORTALITY IN chILD
chILD can be associated with significant mortality15,33
Morbidity and mortality rates vary depending on the specific chILD disorder33
Risk factors for early mortality in chILD
Health-related quality of life (HRQoL) in chILD
HRQoL is significantly impaired in patients with chILD vs. healthy population35
Risk factors for impaired HRQoL in chILD
Caregiver burden in chILD
- Parent HRQoL is impaired, especially mental functioning, which is lower when perception of their child’s HRQoL is also low36
- 74%–91% of parents report anxiety or depression22,37
- 44% of parents report productivity loss at the time of initial presentation of chILD, which continues to be the case in 33% of parents after 5 years of follow-up38
- * The period prevalence range of chILD in Europe was derived for children aged 0 to 18 years: 1.3 cases per million in Germany (2009);4 3.6 cases per million in the UK and Ireland (2002);5 and 46.5 cases per million in Spain (2022).6
- AE-ILD, acute exacerbation of interstitial lung disease; chILD, childhood interstitial lung disease; ES, effect size; HRCT, high-resolution computed tomography; HRQoL, health-related quality of life; ILD, interstitial lung disease; PedsQL, Pediatric Quality of Life Inventory; SpO2, peripheral oxygen saturation.
- Deutsch GH, Young LR, Deterding RR, et al. Diffuse lung disease in young children: application of a novel classification scheme. Am J Respir Crit Care Med. 2007;176:1120–1128.
- Griese M, Irnstetter A, Hengst M, et al. Categorizing diffuse parenchymal lung disease in children Rare pulmonary diseases. Orphanet Journal of Rare Diseases. 2015;10(1):1–6.
- Griese M, Seidl E, Hengst M, et al. International management platform for children’s interstitial lung disease (chILD-EU). Thorax. 2018;73:231–239.
- Griese M, et al. Incidence and classification of pediatric diffuse parenchymal lung diseases in Germany. Orphanet J Rare Dis. 2009;4:26.
- Dinwiddie R, Sharief N, Crawford O. Idiopathic interstitial pneumonitis in children: A national survey in the United Kingdom and Ireland. Pediatric Pulmonology. 2002;34(1):23–29.
- Torrent-Vernetta A, Gaboli M, Castillo-Corullón S, et al. Incidence and prevalence of children's diffuse lung disease in Spain. Archivos de Bronconeumología. 2022;58(1):22–29.
- Ferraro VA, Zanconato S, Zamunaro A, et al. Children’s interstitial and diffuse lung diseases (chILD) in 2020. Children. 2020;7(12):280.
- Nathan N, Berdah L, Delestrain C, et al. Interstitial lung diseases in children. La Presse Médicale. 2020;49(2):103909.
- Hime NJ, Zurynski Y, Fitzgerald D, et al. Childhood interstitial lung disease: A systematic review. Pediatric Pulmonology. 2015;50(12):1383–1392.
- Seidl E, Schwerk N, Carlens J, et al. Acute exacerbations in children’s interstitial lung disease. Thorax. 2022;77(8):799–804.
- Abdelhady SG, Fouda EM, Shaheen MA, et al. Spectrum of childhood interstitial and diffuse lung diseases at a tertiary hospital in Egypt. European Respiratory Journal Open Research. 2021;7(2).
- Thomasson AD, Abou Taam A, Berteloot L, et al. Sleep in children and young adults with interstitial and diffuse lung disease. Sleep Medicine. 2021;80:23–29.
- Tural DA, Emiralioglu N, Ozsezen B, et al. Clinical spectrum of children with interstitial pneumonia with autoimmune features. Respiratory Medicine. 2021;187:106566.
- Hafezi N, Heimberger MA, Lewellen KA, et al. Lung biopsy in children's interstitial and diffuse lung disease: Does it alter management? Pediatric Pulmonology. 2020;55(4):1050–1060.
- Cunningham S, Graham C, MacLean M, et al. One-year outcomes in a multicentre cohort study of incident rare diffuse parenchymal lung disease in children (ChILD). Thorax. 2020;75(2):172–175.
- Witt S, Buchvald F, Bush A, et al. Disease characteristics of a large European registry cohort of children with Interstitial lung diseases (chILD). Atemwegs- und Lungenkrankheiten. 2019;45(2):89–90.
- Niemitz M, Schwerk N, Goldbeck L, et al. Development and validation of a health-related quality of life questionnaire for pediatric patients with interstitial lung disease. Pediatric Pulmonology. 2018;53(7):954–963.
- Young L, Nevel R, Casey A, et al. A national registry for childhood interstitial and diffuse lung diseases in the United States. European Respiratory Journal. 2018;52.
- Saddi V, Beggs S, Bennetts B, et al. Childhood interstitial lung diseases in immunocompetent children in Australia and New Zealand: A decade’s experience. Orphanet Journal of Rare Diseases. 2017;12(1):1–9.
- Hamed EA, El-Saied MM, Saad K, et al. Molecular mechanisms underlying fibrosis and elastin destruction in childhood interstitial lung diseases. Pathophysiology. 2016;23(4):275–283.
- Fan LL, Dishop MK, Galambos C, et al. Diffuse lung disease in biopsied children 2 to 18 years of age application of the chILD classification scheme. Annals of the American Thoracic Society. 2015;12(10):1498–1505.
- Gilbert C, Bush A, Cunningham S. Childhood interstitial lung disease: Family experiences. Pediatric Pulmonology. 2015;50(12):1301–1303.
- Xu D, Chen Z, Chen H, et al. Application of clinico-radiologic-pathologic diagnosis of diffuse parenchymal lung diseases in children in China. PLoS ONE. 2015;10(1).
- Sankar J, Pillai MS, Jeeva Sankar M, et al. Clinical profile of interstitial lung disease in Indian Children. Indian Pediatrics. 2013;50(1):127–133.
- Paiva MAS, Amaral SM. Chronic interstitial lung disease in children. Jornal de Pediatria. 2007;83:233–240.
- Vijayasekaran D, Giridhar S, Gowrishankar N, et al. Pediatric interstitial lung disease. Indian Pediatr. 2006;43(10):899–903.
- Clement A, Allen J, Corrin B, et al. Task force on chronic interstitial lung disease in immunocompetent children. European Respiratory Journal. 2004;24(4):686–697.
- Rice A, Tran-Dang M, Bush A, et al. Diffuse lung disease in infancy and childhood: Expanding the chILD classification. Histopathology. 2013;63:743–755.
- Nathan N, Sileo C, Thouvenin G, et al. Pulmonary Fibrosis in Children. J Clin Med. 8 (1312): 2019.
- Brauner M, et al. Imagerie des pneumopathies infiltrantes diffuses. Press Med. 2010;39:73–84.
- Fan LL, Kozinetz C. Factors Influencing Survival in Children with Chronic Interstitial Lung Disease. Am J Respir Crit Care Med. 1997;156(3 Pt 1):939–42.
- Kurland G, et al. An official ATS clinical practice guideline: classification, evaluation, and management of childhood interstitial lung disease (chILD) in infancy. Am J Respir Crit Care Med. 2013;188:376–94.
- Deterding RR, DeBoer EM, Cidon MJ, et al. Approaching clinical trials in childhood interstitial lung disease and pediatric pulmonary fibrosis. American Journal of Respiratory and Critical Care Medicine. 2019;200(10):1219–1227.
- Bromley S, Vizcaya D. Pulmonary hypertension in childhood interstitial lung disease: A systematic review of the literature. Pediatric Pulmonology. 2017;52(5):689–698.
- Lauby C, Boelle PY, Abou Taam R, et al. Health-related quality of life in infants and children with interstitial lung disease. Pediatric Pulmonology. 2019;54(6):828–836.
- Kelada L, Wakefield C, Vidic N, et al. Genomic testing for children with interstitial and diffuse lung disease (chILD): Parent satisfaction, understanding and health-related quality of life. BMJ open respiratory research. 2022;9(1):e001139.
- Boyd J, Cunningham S, Schwerk N, et al. Challenges facing parents/caregivers of children with interstitial lung disease (chILD): International survey. Eur Respiratory Society; 2018.
- Seidl E, Schwerk N, Carlens J, et al. Healthcare resource utilisation and medical costs for children with interstitial lung diseases (chILD) in Europe. Thorax. 2022;77(8):781–789.