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

Childhood interstitial lung disease


Prevalence of chILD in Europe is estimated to be between 1.3 and 46.5 cases per million children*4–6

Prevalence of chILD by age group in Spain


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

the most common signs and symptoms of child

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

heart disease

Heart disease 
nervous system disease

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


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

  • Younger age at baseline15
  • Fan severity score24,31
  • Pulmonary hypertension34
  • Almost mature / immature gestational age10
  • High baseline severity24
  • Low baseline SpO215
  • Immunocompromised status21
  • Use of mechanical ventilation21


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

  • Younger age17
  • Higher disease severity17,35
  • ≥1 incident(s) of AE-ILD10
  • Extra-pulmonary localization of disease35
  • Use of non-pharmaceutical therapies17,35
  • Lower mental functioning of parents36


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.

  1. 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.
  2. 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.
  3. Griese M, Seidl E, Hengst M, et al. International management platform for children’s interstitial lung disease (chILD-EU). Thorax. 2018;73:231–239.
  4. Griese M, et al. Incidence and classification of pediatric diffuse parenchymal lung diseases in Germany. Orphanet J Rare Dis. 2009;4:26.
  5. 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.
  6. 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.
  7. Ferraro VA, Zanconato S, Zamunaro A, et al. Children’s interstitial and diffuse lung diseases (chILD) in 2020. Children. 2020;7(12):280.
  8. Nathan N, Berdah L, Delestrain C, et al. Interstitial lung diseases in children. La Presse Médicale. 2020;49(2):103909.
  9. Hime NJ, Zurynski Y, Fitzgerald D, et al. Childhood interstitial lung disease: A systematic review. Pediatric Pulmonology. 2015;50(12):1383–1392.
  10. Seidl E, Schwerk N, Carlens J, et al. Acute exacerbations in children’s interstitial lung disease. Thorax. 2022;77(8):799–804.
  11. 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).
  12. 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.
  13. Tural DA, Emiralioglu N, Ozsezen B, et al. Clinical spectrum of children with interstitial pneumonia with autoimmune features. Respiratory Medicine. 2021;187:106566.
  14. 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.
  15. 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.
  16. 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.
  17. 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.
  18. 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.
  19. 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.
  20. 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.
  21. 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.
  22. Gilbert C, Bush A, Cunningham S. Childhood interstitial lung disease: Family experiences. Pediatric Pulmonology. 2015;50(12):1301–1303.
  23. 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).
  24. 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.
  25. Paiva MAS, Amaral SM. Chronic interstitial lung disease in children. Jornal de Pediatria. 2007;83:233–240.
  26. Vijayasekaran D, Giridhar S, Gowrishankar N, et al. Pediatric interstitial lung disease. Indian Pediatr. 2006;43(10):899–903.
  27. 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.
  28. 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.
  29. Nathan N, Sileo C, Thouvenin G, et al. Pulmonary Fibrosis in Children. J Clin Med. 8 (1312): 2019.
  30. Brauner M, et al. Imagerie des pneumopathies infiltrantes diffuses. Press Med. 2010;39:73–84.
  31. 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.
  32. 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.
  33. 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.
  34. Bromley S, Vizcaya D. Pulmonary hypertension in childhood interstitial lung disease: A systematic review of the literature. Pediatric Pulmonology. 2017;52(5):689–698.
  35. 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.
  36. 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.
  37. 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.
  38. 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.

Resources for patients