12 MIN READ
Childhood interstitial lung disease
Learn about childhood interstitial lung disease (chILD) and discover how you can best support the child you care for following diagnosis.
What is chILD?
Childhood interstitial lung disease (chILD) is a group of 200 rare lung conditions that is estimated to effect between 1 and 47 per million children in Europe.1-7
chILD affects the space around the air sacs (tiny branches of air tubes) in the lungs of children.7,8 Some chILDs involve pulmonary fibrosis (thickening or scarring on the lungs). These disorders have various causes and affect children in different ways. Common symptoms to look out for include coughing, difficulties breathing or quick breathing.7,8
Examples of different chILDs:6
Alveolar surfactant disorders
(abnormalities or functional issues affecting the lung surface)
Disorders of the air sac surfaces in the lungs resulting in impaired air transport
(disorders caused by genetic mutations)
Unexplained conditions in young children and newborns
Immunodeficiency diseases
(disorders that affect the immune system)
Exposure-related ILDs
(caused by exposure to hazards, such as asbestosis and hypersensitivity)
HOW COMMON IS chILD?
In Europe, chILD is estimated to affect between 1 and 47 per million children.1-3 The majority of chILD cases occur in children below the age of 1.3 One study of 90 children with chILD revealed the average age of children with chILD was 6.8 years old.9 However, it is important to note that although chILD is rare in older children,10 it can occur up to the age of 18.4-7
chILD varies greatly, some symptoms are mild whereas others can potentially be very serious.11 However, connecting specific symptoms to a particular chILD can be challenging. This is because although there are a lot of different possible symptoms, there is similarity in how they appear in each chILD.11
Symptoms of chILD may include:11-30
Respiratory retraction (difficulty breathing)
Wheezing, often misdiagnosed as a symptom of asthma
Being comparatively underweight for age and sex
Enlargement of toes or fingertips
Blue or grey skin or lips
Coughing
Rapid breathing
Shortness of breath
Difficulties with physical activity
Children with chILD may also have other conditions affecting the heart, nervous system, the musculoskeletal system (bones, cartilage, ligaments, tendons and connective tissues), or skin.19-21,24
If a child has chILD, it is crucial for their doctor to determine the specific type and whether they have pulmonary fibrosis.10 This is so the doctor can provide the appropriate treatment. To reach a diagnosis the doctor will need to review your child’s medical history, and consider any family medical history. The doctor may request permission to carry out pulmonary (lung) functions tests.31 This will give them a better understanding of your child’s lung health.
The doctor may also be interested in carrying out genetic testing (in order to potentially identify alveolar surfactant disorders), chest computed tomography (CT) (a scan that provides more detailed imaging than normal x-ray are capable of providing), or lung biopsy (a procedure which involves removing samples of lung tissues).31,32
Although, there are several steps to take to reach a diagnosis, these are necessary due to the rarity and variety of chILD.31 It can take a lot of time before your child’s doctor can determine a definitive diagnosis.31 It is also important for them to characterise any other conditions or symptoms, to make sure any necessary treatments are prescribed.31
How chILD affects each child can vary over time,11 and some children with certain types of chILD may even show signs of improvement over a period of 2 to 20 years.21,23,28,30 Your child’s doctor will want to see how chILD affects them over time. They may observe calorie intake, weight and height.32 They may also ask for permission to carry out further physical examinations, walking tests and chest CT (a type of X-ray that provides a more detailed image) to assess whether your child’s lung health has improved.32
Managing chILD, both physically and mentally, can be challenging for both you and your child.21 Your child’s doctor can help you find support for chILD.32 This includes care resources and chILD support groups32, where you or your child can connect with others who know what you are going through. Remember you are not alone in this.
QUICK TIP
Keeping a diary of your child’s symptoms and mood, as well as your own, can be helpful. It allows you to track how both of you are coping with chILD, other conditions and any treatments. It enables you to note down anything you want to share with the doctor, or any questions you want to ask them.
Once chILD is diagnosed, your child’s doctor will discuss possible treatment options.31 After a specific treatment is prescribed, they will closely track your child’s response to ensure this approach is suitable.32
Non-pharmacological interventions and supportive therapies such as oxygen therapy and nutritional support may be recommended by your child’s doctor to manage chILD.11,21,32 Your child’s doctor will also be able to treat other existing comorbidities or conditions.32 In rare, but serious cases where chILD has progressed significantly, lung transplant may be considered.11
In addition to the information from your doctor, there are some useful websites that you can find in the Additional Resources tab on the right-hand side of this page.
Caring for a child with chILD can impact your life as a parent or caregiver.25,33,34 It can require a significant amount of time to meet your child’s care requirements.34 This responsibility could disrupt your personal and professional life and affect your mental and emotional wellbeing.25,33-35 If you need support, speak to your child’s doctor. They will be able tell you what resources are available in your area.
Key takeaways
chILD is rare, with onset usually before or at 1 year of age, but it can affect children up to 18 years old
chILD symptoms vary greatly from mild to serious, so it is important for your child’s doctor to reach a correct diagnosis of the type of chILD. This can be done by looking at medical history and carrying out physical examination and other tests
chILD can affect both yours and your child’s quality of life. There are non-pharmacological interventions and supportive therapies available, as well as range of caregiver resources to help your child and you cope with the burden of chILD. Look at the links on the right-hand side for further information
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.
Griese M, et al. Orphanet J Rare Dis. 2009;4:26.
Torrent-Vernetta A, Gaboli M, Castillo-Corullón S, Mondéjar-López P, Santiago VS, Costa-Colomer J, et al. Incidence and prevalence of children's diffuse lung disease in Spain. Archivos de Bronconeumología. 2022;58(1):22-29.
Deutsch GH, et al. Am J Respir Crit Care Med. 2007;176:1120-28.
Griese M, Irnstetter A, Hengst M, Burmester H, Nagel F, Ripper J, et al. Categorizing diffuse parenchymal lung disease in children. Orphanet Journal of Rare Diseases. 2015;10(1):1-6.
Griese M, et al. Thorax. 2018;73:231-9.
Deterding RR, DeBoer EM, Cidon MJ, Robinson TE, Warburton D, Deutsch GH, et al. Approaching clinical trials in childhood interstitial lung disease and pediatric pulmonary fibrosis. American Journal of Respiratory and Critical Care Medicine. 2019;200(1).
Cunningham S, et al. Lancet Child Adolesc Health. 2019;3:568-77.
Sankar J, Pillai MS, Jeeva Sankar M, Lodha R, Kabra SK. Clinical profile of interstitial lung disease in Indian Children. Indian Pediatrics. 2013;50(1):127-133.
Lee EY. Pediatric interstitial (diffuse) lung disease. Imaging in Pediatric Pulmonology. 2019:145-97.
Ferraro VA, Zanconato S, Zamunaro A, Carraro S. Children’s interstitial and diffuse lung diseases (chILD) in 2020. Children. 2020;7(12):280.
Nathan N, Berdah L, Delestrain C, Sileo C, Clement A. Interstitial lung diseases in children. La Presse Médicale. 2020;49(2):103909.
Hime NJ, Zurynski Y, Fitzgerald D, Selvadurai H, Phu A, Deverell M, et al. Childhood interstitial lung disease: A systematic review. Pediatric Pulmonology. 2015;50(12):1383-1392.
Seidl E, Schwerk N, Carlens J, Wetzke M, Emiralioğlu N, Kiper N, et al. Acute exacerbations in children’s interstitial lung disease. Thorax. 2022a;77(8):799-804.
Abdelhady SG, Fouda EM, Shaheen MA, Ghazal FA, Mostafa AM, Osman AM, 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, Khirani S, Griffon L, Hadchouel A, 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, Sunman B, Buyuksahin HN, Guzelkas I, et al. Clinical spectrum of children with interstitial pneumonia with autoimmune features. Respiratory Medicine. 2021;187:106566.
Hafezi N, Heimberger MA, Lewellen KA, Maatman T, Montgomery GS, Markel TA. 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, Aurora P, Ashworth M, Barbato A, 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, Cunningham S, Kiper N, Lange J, 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, Griese M. 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, Fishman M, Welsh S, Liptzin D, 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, Harrison J, Hime N, Kapur N, 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, Yousef HAZ, Mohamed AO, Sabry D. Molecular mechanisms underlying fibrosis and elastin destruction in childhood interstitial lung diseases. Pathophysiology. 2016;23(4):275-283.
Fan LL, Dishop MK, Galambos C, Askin FB, White FV, Langston 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, Huang R, Zhao S, Liu X, et al. Application of clinico-radiologic-pathologic diagnosis of diffuse parenchymal lung diseases in children in China. PLoS ONE. 2015;10(1).
Paiva MAS, Amaral SM. Chronic interstitial lung disease in children. Jornal de Pediatria. 2007;83:233-240.
Vijayasekaran D, Giridhar S, Gowrishankar N, Nedunchelian K, Senguttuvan M. Pediatric interstitial lung disease. Indian Pediatr. 2006;43(10):899-903.
Clement A, Allen J, Corrin B, Dinwiddie R, le Pointe HD, Eber E, et al. Task force on chronic interstitial lung disease in immunocompetent children. European Respiratory Journal. 2004;24(4):686-697.
Nathan N, Griese M, Michel K, et al. Diagnostic workup of childhood interstitial lung disease. Eur Respir Rev. 2023;32(167):220188.
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.
Boyd J, Cunningham S, Schwerk N, Gilbert C, Powell P, Schamberger M. Challenges facing parents/caregivers of children with interstitial lung disease (chILD): International survey. Eur Respiratory Society; 2018.
Seidl E, Schwerk N, Carlens J, Wetzke M, Cunningham S, Emiralioğlu N, et al. Healthcare resource utilisation and medical costs for children with interstitial lung diseases (chILD) in Europe. Thorax. 2022b;77(8):781-789.
Kelada L, Wakefield C, Vidic N, Armstrong DS, Bennetts B, Boggs K, 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.and Ireland. Pediatric Pulmonology. 2002;34(1):23-29.
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