Interstitial lung disease in children
Infantile
interstitial lung disease is a group of rare lung diseases affecting infants,
children and adolescents.
All forms of
childhood interstitial lung disease cause damage to the child's lungs, causing
them to stop working properly.
Many types of
childhood interstitial lung disease affect the interstitium, the thin tissue
between the tiny air sacs and blood vessels in the lungs. Some types of
childhood interstitial lung disease also affect other areas of the lungs.
Doctors have only
begun to understand this condition in the last few years, and they don't know
much. For example, they do not know how many children have interstitial lung
disease during childhood.
Among children with
childhood interstitial lung disease, some are born with it, and some develop it
later in childhood. Adults can also get interstitial lung disease, but the
causes and outcomes are often different.
Causes
Interstitial lung
disease in children can occur without a known cause. On the other hand, certain
genes, toxins, or other diseases may be involved.
Some possible
reasons:
- Inherited diseases. Diseases that cause problems with surfactant—the fluid
in your lungs that helps your baby breathe—can be passed down through the
genes.
- Problems with the immune system: Some problems with the immune system make
it difficult for children to fight diseases.
- Autoimmune diseases: These occur when your child's immune system mistakenly
attacks healthy tissues. Inflammatory bowel disease and collagen vascular
disease are two autoimmune diseases commonly associated with interstitial lung
disease in children.
- Infection: Some children develop interstitial lung disease during childhood after a cold or a virus.
- Birth defects: Babies can be born with birth defects that cause lung
problems.
- Aspiration: When you inhale food, liquids, or vomit into your lungs, it can cause damage. Aspiration often affects children with swallowing problems or a condition called gastroesophageal reflux disease.
- Cancer treatment: Therapies such as radiation and chemotherapy can lead
to interstitial lung disease in children.
- Environmental triggers: Chemicals and mold can irritate your child's lungs.
- Surgery: Lung transplant or bone marrow transplant can cause some cases of childhood interstitial lung disease.
Types
There are different
types of childhood interstitial lung disease. Many have long and difficult to
pronounce names. Although they are all considered rare diseases, some forms are
more common in certain age groups.
Pediatric
interstitial lung diseases that commonly affect children include:
- Surfactant dysfunction mutations
- Developmental disorders, such as alveolar capillary dysplasia
- Lung growth abnormalities
- Neuroendocrine cell hyperplasia of infancy
- Pulmonary interstitial glycogenosis
The most common types
of childhood interstitial lung disease in children and adolescents are:
- Idiopathic interstitial pneumonia: This category includes cryptogenic
organizing pneumonia, acute interstitial pneumonia, nonspecific interstitial
pneumonia, desquamative interstitial pneumonia, and lymphocytic interstitial
pneumonia.
- Other primary disorders: These disorders can be alveolar hemorrhage syndromes,
aspiration syndromes, hypersensitivity pneumonitis, bronchiolitis obliterans,
eosinophilic pneumonia, pulmonary alveolar proteinosis, pulmonary infiltrates
with eosinophilia, pulmonary lymphatic vessels (lymphangiomatosis,
lymphangiectasia) or pulmonary diseases.
- Interstitial lung disease associated with systemic pathological processes: for
example, connective tissue disease, histiocytosis, malignant lung disease,
sarcoidosis and storage disease.
- Immune system disorders: This group includes opportunistic infections,
treatment-related disorders, lung diseases associated with lung and bone marrow
transplantation, and diffuse alveolar damage of unknown cause.
Symptoms
The signs and
symptoms of interstitial lung disease in children often depend on the type of
disease and its severity. They may include:
- Difficulty breathing or shortness of breath
- Fast or noisy breathing
- Wheezing
- Coughing or chest congestion
- Repeated bouts of pneumonia or bronchitis
- Low oxygen levels
- Failure to gain weight or grow in height
Interstitial disease in adults and children
Some children who
have had interstitial lung disease in childhood will have the disease
throughout their lives, so technically it can occur in both children and
adults.
But when an adult is
diagnosed with interstitial lung disease, doctors usually consider it to be a
completely different disease than interstitial lung disease in children.
Children with
childhood interstitial lung disease should see a pediatric pulmonologist, not
an adult doctor.
Diagnosis
Diagnosis of
interstitial lung disease in children is often difficult. Because each type is
different, the methods your doctor uses will vary.
Tests that help
diagnose interstitial lung disease in children include:
- Chest x-ray or CT scan: These imaging procedures use x-rays to take pictures of
your baby's lungs.
- Lung function tests: Doctors measure the number of breaths children take to
see how well their lungs are working.
- Blood tests: Blood tests are sometimes used to look for abnormal
genes.
- Bronchoalveolar lavage: In this procedure, the doctor injects salt water
through a tube into your child's lungs to see certain types of cells. This can
help detect lung damage, aspiration, infection, or airway problems.
- Lung biopsy: The surgeon removes a small piece of lung tissue for
examination in the laboratory.
Treatment
Very little research
has been done on how to treat interstitial lung disease in children. But some
treatments can help children's lungs work better, relieve symptoms, or just
make them feel better.
Your doctor may
recommend:
- Medicines:
Steroids reduce inflammation in the lungs, antimicrobials treat infections, and
bronchodilators help relax the muscles around the airways.
- Oxygen: More oxygen can help children breathe better and give their hearts a rest.
- Nutrition: A weight-gain diet may benefit some children with childhood interstitial lung disease.
- Pulmonary rehab and exercises: Special therapies aim to reduce congestion
and improve lung function (your doctor may call this "lung
function").
- Breathing devices: Devices called ventilators can help children breathe
better.
- Lung transplant: This may be an option for children with severe or life-threatening cases of childhood interstitial lung disease. To date, interstitial lung disease in children does not appear to have recurred in operated children.