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| Meta Title | Childhood Interstitial Lung Disease | Symptoms & Treatment |
| Meta Description | Interstitial lung disease (ILD) is a group of disorders that affect the lungs. Learn about symptoms, diagnosis, types and treatment. |
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Interstitial lung disease (ILD) includes a large group of disorders that affect the interstitium part of the lung. The interstitium lies between the alveolar sacs in the lung where gas exchange occurs. The small air tubes (called bronchioles) may also be affected.
In ILD, the interstitium can be abnormal due to:
Scar tissue (called pulmonary fibrosis)
Build-up of too many or different cells
Injuries or changes to the lung structure
ILD is uncommon in children.
What are the Signs and Symptoms of ILD?
Fast breathing (called tachypnea)
Sinking in of the areas below the ribs, between the ribs, and in the neck with breathing (called retractions)
Abnormally low amount of oxygen in the blood (called low oxygen saturations)
Crackle sounds in the lungs that are heard with a stethoscope
Chronic cough
Shortness of breath, which might include trouble with exercise or physical activity
Problems growing and gaining weight
Reflux
(food or drink travels from the stomach up the esophagus to the throat or mouth)
Many episodes of
pneumonia
Â
Chest CT scans that show abnormalities throughout both lungs
Most children with ILD show respiratory symptoms all the time, even when there is not an infection present. Children with ILD may have only some of the symptoms.
How is ILD Diagnosed?
Visits with a lung doctor (called a pulmonologist)
Tests may be done to make sure your child does not have
cystic fibrosis
, a lung infection, heart problems or other health problems
Testing for ILD may include one or all of the following:
Blood tests
Bronchoscopy
High-resolution chest CT scan
Pulmonary function tests or infant pulmonary function tests
Lung biopsy
Decisions about specific testing may depend on:
The age of the child
Other medical problems
How long symptoms have been there
How bad the symptoms are
Whether the symptoms are getting better or getting worse
History of ILD in family membersÂ
What Disorders are Associated with ILD?
Connective-tissue diseases (also called collagen vascular disease)
Pulmonary hemorrhage syndromes, including capillaritis, Goodpasture’s syndrome, or vasculitis
Langerhans cell histiocytosis
Sarcoidosis
Storage disorders
Lymphoproliferative disorders
Eosinophilic disorders
Neurocutaneous Syndromes
What are the Types of ILD in Children?
Some types of ILD common for infants and young children include:
Disorders of lung development and lung growth:
Alveolar capillary dysplasia with misalignment of the pulmonary veins
Pulmonary hypoplasia
Bronchopulmonary dysplasia (BPD)
Pulmonary interstitial glycogenosis (PIG)
Neuroendocrine cell hyperplasia of infancy (NEHI)
Disorders of surfactant production or alveolar proteinosis due to SP-B genetic mutations, SP-C genetic mutations, ABCA-3 genetic mutations, NKX2-1 genetic mutations, GMCSF receptor deficiency, or lysinuric protein intolerance, or others
Certain surfactant disorders can also occur in older children and adults.
Other types of ILD can start at any age.
Some ILD disorders occur along with other medical problems, meaning parts of the body other than the lung are also likely to be affected.
Some ILD disorders may be due to problems with the function of the immune system.
Autoimmune pulmonary alveolar proteinosis due to auto-antibodies to GM-CSF
Follicular bronchiolitis / lymphocytic interstitial pneumonitis, constrictive bronchiolitis, or lymphoproliferative disorder are often related to immunodeficiency
Some ILD disorders may occur due to lung injuries.
Aspiration
Hypersensitivity pneumonitis
Bronchiolitis obliterans
Some disorders are due to problems with blood vessels or lymphatic vessels and can look like ILD.
Lymphangiomatosis, lymphangiectasia, and
lymphatic malformations
Â
Veno-occlusive disease
Pulmonary arterial vasculopathy
How is ILD Treated?
Different treatments are used for different types of ILD. Understanding the cause is important to decide on treatment. Some treatments may include:
More oxygen (not every child needs it)
More nutrition to help gain weight and growth
Flu shot every year to lower the chance of getting infections
Different medications, depending on the type of ILD
Last Updated
10/2025
Reviewed By
Laura Bellew, RN
Meet the specialists in the Pediatric Rare Lung Diseases Program. |
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# Health LibraryInterstitial Lung Disease
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[Close](https://www.cincinnatichildrens.org/health/i/ild)
### [Rare Lung Diseases](https://www.cincinnatichildrens.org/service/r/rare-lung)
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## What is Interstitial Lung Disease?
Interstitial lung disease (ILD) includes a large group of disorders that affect the interstitium part of the lung. The interstitium lies between the alveolar sacs in the lung where gas exchange occurs. The small air tubes (called bronchioles) may also be affected.
In ILD, the interstitium can be abnormal due to:
- Scar tissue (called pulmonary fibrosis)
- Build-up of too many or different cells
- Injuries or changes to the lung structure
ILD is uncommon in children.
### What are the Signs and Symptoms of ILD?
- Fast breathing (called tachypnea)
- Sinking in of the areas below the ribs, between the ribs, and in the neck with breathing (called retractions)
- Abnormally low amount of oxygen in the blood (called low oxygen saturations)
- Crackle sounds in the lungs that are heard with a stethoscope
- Chronic cough
- Shortness of breath, which might include trouble with exercise or physical activity
- Problems growing and gaining weight
- [Reflux](https://www.cincinnatichildrens.org/health/g/ger-child "Learn more about Reflux.") (food or drink travels from the stomach up the esophagus to the throat or mouth)
- Many episodes of [pneumonia](https://www.cincinnatichildrens.org/health/p/pneumonia "Learn more about pneumonia.")
- Chest CT scans that show abnormalities throughout both lungs
Most children with ILD show respiratory symptoms all the time, even when there is not an infection present. Children with ILD may have only some of the symptoms.
### How is ILD Diagnosed?
- Visits with a lung doctor (called a pulmonologist)
- Tests may be done to make sure your child does not have [cystic fibrosis](https://www.cincinnatichildrens.org/health/c/cystic-fibrosis "Learn more about cystic fibrosis."), a lung infection, heart problems or other health problems
Testing for ILD may include one or all of the following:
- Blood tests
- [Bronchoscopy](https://www.cincinnatichildrens.org/service/b/bronchoscopy "Learn more about Bronchoscopy.")
- High-resolution chest CT scan
- Pulmonary function tests or infant pulmonary function tests
- Lung biopsy
Decisions about specific testing may depend on:
- The age of the child
- Other medical problems
- How long symptoms have been there
- How bad the symptoms are
- Whether the symptoms are getting better or getting worse
- History of ILD in family members
### What Disorders are Associated with ILD?
- Connective-tissue diseases (also called collagen vascular disease)
- Pulmonary hemorrhage syndromes, including capillaritis, Goodpasture’s syndrome, or vasculitis
- [Langerhans cell histiocytosis](https://www.cincinnatichildrens.org/health/l/langerhans-cell-histiocytosis-lch "Learn more about Langerhans cell histiocytosis.")
- Sarcoidosis
- Storage disorders
- Lymphoproliferative disorders
- Eosinophilic disorders
- Neurocutaneous Syndromes
### What are the Types of ILD in Children?
#### Some types of ILD common for infants and young children include:
- Disorders of lung development and lung growth:
- Alveolar capillary dysplasia with misalignment of the pulmonary veins
- Pulmonary hypoplasia
- [Bronchopulmonary dysplasia (BPD)](https://www.cincinnatichildrens.org/health/b/bronchopulmonary-dysplasia "Learn more about Bronchopulmonary dysplasia (BPD).")
- [Pulmonary interstitial glycogenosis (PIG)](https://www.cincinnatichildrens.org/health/p/pig "Learn more about Pulmonary interstitial glycogenosis (PIG).")
- Neuroendocrine cell hyperplasia of infancy (NEHI)
- Disorders of surfactant production or alveolar proteinosis due to SP-B genetic mutations, SP-C genetic mutations, ABCA-3 genetic mutations, NKX2-1 genetic mutations, GMCSF receptor deficiency, or lysinuric protein intolerance, or others
- Certain surfactant disorders can also occur in older children and adults.
Other types of ILD can start at any age.
Some ILD disorders occur along with other medical problems, meaning parts of the body other than the lung are also likely to be affected.
#### Some ILD disorders may be due to problems with the function of the immune system.
- Autoimmune pulmonary alveolar proteinosis due to auto-antibodies to GM-CSF
- Follicular bronchiolitis / lymphocytic interstitial pneumonitis, constrictive bronchiolitis, or lymphoproliferative disorder are often related to immunodeficiency
#### Some ILD disorders may occur due to lung injuries.
- [Aspiration](https://www.cincinnatichildrens.org/health/c/chronic-aspiration "Learn more about Aspiration.")
- Hypersensitivity pneumonitis
- Bronchiolitis obliterans
#### Some disorders are due to problems with blood vessels or lymphatic vessels and can look like ILD.
- Lymphangiomatosis, lymphangiectasia, and [lymphatic malformations](https://www.cincinnatichildrens.org/health/l/lymphatic-malformations "Learn more about lymphatic malformations.")
- Veno-occlusive disease
- Pulmonary arterial vasculopathy
### How is ILD Treated?
Different treatments are used for different types of ILD. Understanding the cause is important to decide on treatment. Some treatments may include:
- More oxygen (not every child needs it)
- More nutrition to help gain weight and growth
- Flu shot every year to lower the chance of getting infections
- Different medications, depending on the type of ILD
**Last Updated** 10/2025
**Reviewed By** Laura Bellew, RN
[](https://www.cincinnatichildrens.org/service/r/rare-lung/team)
Meet the specialists in the Pediatric Rare Lung Diseases Program.
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Interstitial lung disease (ILD) includes a large group of disorders that affect the interstitium part of the lung. The interstitium lies between the alveolar sacs in the lung where gas exchange occurs. The small air tubes (called bronchioles) may also be affected.
In ILD, the interstitium can be abnormal due to:
- Scar tissue (called pulmonary fibrosis)
- Build-up of too many or different cells
- Injuries or changes to the lung structure
ILD is uncommon in children.
### What are the Signs and Symptoms of ILD?
- Fast breathing (called tachypnea)
- Sinking in of the areas below the ribs, between the ribs, and in the neck with breathing (called retractions)
- Abnormally low amount of oxygen in the blood (called low oxygen saturations)
- Crackle sounds in the lungs that are heard with a stethoscope
- Chronic cough
- Shortness of breath, which might include trouble with exercise or physical activity
- Problems growing and gaining weight
- [Reflux](https://www.cincinnatichildrens.org/health/g/ger-child "Learn more about Reflux.") (food or drink travels from the stomach up the esophagus to the throat or mouth)
- Many episodes of [pneumonia](https://www.cincinnatichildrens.org/health/p/pneumonia "Learn more about pneumonia.")
- Chest CT scans that show abnormalities throughout both lungs
Most children with ILD show respiratory symptoms all the time, even when there is not an infection present. Children with ILD may have only some of the symptoms.
### How is ILD Diagnosed?
- Visits with a lung doctor (called a pulmonologist)
- Tests may be done to make sure your child does not have [cystic fibrosis](https://www.cincinnatichildrens.org/health/c/cystic-fibrosis "Learn more about cystic fibrosis."), a lung infection, heart problems or other health problems
Testing for ILD may include one or all of the following:
- Blood tests
- [Bronchoscopy](https://www.cincinnatichildrens.org/service/b/bronchoscopy "Learn more about Bronchoscopy.")
- High-resolution chest CT scan
- Pulmonary function tests or infant pulmonary function tests
- Lung biopsy
Decisions about specific testing may depend on:
- The age of the child
- Other medical problems
- How long symptoms have been there
- How bad the symptoms are
- Whether the symptoms are getting better or getting worse
- History of ILD in family members
### What Disorders are Associated with ILD?
- Connective-tissue diseases (also called collagen vascular disease)
- Pulmonary hemorrhage syndromes, including capillaritis, Goodpasture’s syndrome, or vasculitis
- [Langerhans cell histiocytosis](https://www.cincinnatichildrens.org/health/l/langerhans-cell-histiocytosis-lch "Learn more about Langerhans cell histiocytosis.")
- Sarcoidosis
- Storage disorders
- Lymphoproliferative disorders
- Eosinophilic disorders
- Neurocutaneous Syndromes
### What are the Types of ILD in Children?
#### Some types of ILD common for infants and young children include:
- Disorders of lung development and lung growth:
- Alveolar capillary dysplasia with misalignment of the pulmonary veins
- Pulmonary hypoplasia
- [Bronchopulmonary dysplasia (BPD)](https://www.cincinnatichildrens.org/health/b/bronchopulmonary-dysplasia "Learn more about Bronchopulmonary dysplasia (BPD).")
- [Pulmonary interstitial glycogenosis (PIG)](https://www.cincinnatichildrens.org/health/p/pig "Learn more about Pulmonary interstitial glycogenosis (PIG).")
- Neuroendocrine cell hyperplasia of infancy (NEHI)
- Disorders of surfactant production or alveolar proteinosis due to SP-B genetic mutations, SP-C genetic mutations, ABCA-3 genetic mutations, NKX2-1 genetic mutations, GMCSF receptor deficiency, or lysinuric protein intolerance, or others
- Certain surfactant disorders can also occur in older children and adults.
Other types of ILD can start at any age.
Some ILD disorders occur along with other medical problems, meaning parts of the body other than the lung are also likely to be affected.
#### Some ILD disorders may be due to problems with the function of the immune system.
- Autoimmune pulmonary alveolar proteinosis due to auto-antibodies to GM-CSF
- Follicular bronchiolitis / lymphocytic interstitial pneumonitis, constrictive bronchiolitis, or lymphoproliferative disorder are often related to immunodeficiency
#### Some ILD disorders may occur due to lung injuries.
- [Aspiration](https://www.cincinnatichildrens.org/health/c/chronic-aspiration "Learn more about Aspiration.")
- Hypersensitivity pneumonitis
- Bronchiolitis obliterans
#### Some disorders are due to problems with blood vessels or lymphatic vessels and can look like ILD.
- Lymphangiomatosis, lymphangiectasia, and [lymphatic malformations](https://www.cincinnatichildrens.org/health/l/lymphatic-malformations "Learn more about lymphatic malformations.")
- Veno-occlusive disease
- Pulmonary arterial vasculopathy
### How is ILD Treated?
Different treatments are used for different types of ILD. Understanding the cause is important to decide on treatment. Some treatments may include:
- More oxygen (not every child needs it)
- More nutrition to help gain weight and growth
- Flu shot every year to lower the chance of getting infections
- Different medications, depending on the type of ILD
**Last Updated** 10/2025
**Reviewed By** Laura Bellew, RN
[](https://www.cincinnatichildrens.org/service/r/rare-lung/team)
Meet the specialists in the Pediatric Rare Lung Diseases Program.
[](https://www.cincinnatichildrens.org/service/r/rare-lung/contact "Contact the Rare Lung Diseases Program.") |
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