Children's interstitial lung disease

Other Names: ChILD; Primary ILD specific to childhood; Primary interstitial lung disease specific to childhood

Children's interstitial and diffuse lung disease (chILD) is not a single condition, but a group of rare lung diseases found in infants, children and adolescents that can range from mild to severe.These diseases have some similar symptoms, such as chronic cough, rapid breathing, and shortness of breath. These diseases also harm the lungs in similar ways. For example, they damage the tissues that surround the lungs' alveoli (al-VEE-uhl-eye; air sacs) and bronchial tubes (airways). Sometimes these diseases directly damage the air sacs and airways. The various types of chILD can decrease lung function, reduce blood oxygen levels, and disturb the breathing process.

Types

The broad term "childhood interstitial lung disease" (chILD) refers to a group of rare lung diseases that can affect babies, children, and teens. Some of these diseases are more common in certain age groups.

Diseases more common in infancy include:

• Surfactant (sur-FAK-tant) dysfunction mutations
• Developmental disorders, such as alveolar capillary dysplasia
• Lung growth abnormalities
• Neuroendocrine cell hyperplasia of infancy (NEHI)
• Pulmonary interstitial glycogenosis (PIG)
• Diseases more common in children older than 2 years of age and teens include:

Idiopathic interstitial pneumonias:

• Nonspecific interstitial pneumonia
• Cryptogenic organizing pneumonia
• Acute interstitial pneumonia
• Desquamative interstitial pneumonia
• Lymphocytic interstitial pneumonia

Other primary disorders:

• Alveolar hemorrhage syndromes
• Aspiration syndromes
• Hypersensitivity pneumonitis
• Infectious or postinfectious disease (bronchiolitis obliterans)
• Eosinophilic pneumonia
• Pulmonary alveolar proteinosis
• Pulmonary infiltrates with eosinophilia
• Pulmonary lymphatic disorders (lymphangiomatosis, lymphangiectasis)
• Pulmonary vascular disorders (haemangiomatosis)

ILD associated with systemic disease processes:

• Connective tissue diseases
• Histiocytosis
• Malignancy-related lung disease
• Sarcoidosis
• Storage diseases

Disorders of the compromised immune system:

• Opportunistic infection
• Disorders related to therapeutic intervention
• Lung and bone marrow transplant-associated lung diseases
• Diffuse alveolar damage of unknown cause

The various types of chILD can affect many parts of the lungs, including the alveoli (air sacs), bronchial tubes (airways), and capillaries. (Capillaries are the tiny blood vessels that surround the air sacs).

Cause

Researchers don't yet know all of the causes of childhood interstitial lung disease (chILD). Many times, these diseases have no clear cause.

Some conditions and factors that may cause or lead to chILD include:

• Inherited conditions, such as surfactant disorders. Surfactant is a liquid that coats the inside of the lungs. It helps with breathing and may help protect the lungs from bacterial and viral infections.
• Birth defects that cause problems with the structure or function of the lungs.
• Aspiration (as-pih-RA-shun). This term refers to inhaling substances—such as food, liquid, or vomit—into the lungs. Inhaling these substances can injure the lungs. Aspiration may occur in children who have swallowing problems or gastroesophageal (GAS-tro-eh-so-fa-JE-al) reflux disease (GERD). GERD occurs if acid from the stomach backs up into the throat.
• Immune system disorders. The immune system protects the body against bacteria, viruses, and toxins. Children who have immune system disorders aren't able to fight illness and disease as well as children who have healthy immune systems.
• Exposure to substances in the environment that can irritate the lungs, such as molds and chemicals.
• Some cancer treatments, such as radiation and chemotherapy.
• Systemic or autoimmune diseases, such as collagen vascular disease or inflammatory bowel disease. Systemic diseases are diseases that involve many of the body's organs. Autoimmune diseases occur if the body's immune system mistakenly attacks the body's tissues and cells.
• A bone marrow transplant or a lung transplant.

Signs and symptoms

Childhood interstitial lung disease (chILD) has many signs and symptoms because the disease has many forms. Signs and symptoms may include:

• Fast breathing, which also is called tachypnea (tak-ip-NE-ah)
• Labored breathing, which also is called respiratory distress
• Low oxygen levels in the blood, which also is called hypoxemia (hi-POK-se-ah)
• Recurrent coughing, wheezing, or crackling sounds in the chest
• Shortness of breath during exercise (in older children) or while eating (in infants), which also is called dyspnea (disp-NE-ah)
• Poor growth or failure to gain weight
• Recurrent pneumonia or bronchiolitis

Diagnosis

Doctors diagnose childhood interstitial lung disease (chILD) based on a child's medical and family histories and the results from tests and procedures. To diagnose chILD, doctors may first need to rule out other diseases as the cause of a child's symptoms.

Early diagnosis of chILD may help doctors stop or even reverse lung function problems. Often though, doctors find chILD hard to diagnose because:

• There are many types of the disease and a range of underlying causes
• The disease's signs and symptoms are the same as those for many other diseases
• The disease may coexist with other diseases
• Going to a pediatric pulmonologist who has experience with chILD is helpful. A pediatric pulmonologist is a doctor who specializes in diagnosing and treating children who have lung diseases and conditions.

Diagnostic Tests and Procedures No single test can diagnose the many types of chILD. Thus, your child's doctor may recommend one or more of the following tests. For some of these tests, infants and young children may be given medicine to help them relax or sleep.

• A chest x ray. This painless test creates pictures of the structures inside your child's chest, such as the heart, lungs, and blood vessels. A chest x ray can help rule out other lung diseases as the cause of your child's symptoms.
• A high-resolution CT scan (HRCT). An HRCT scan uses x rays to create detailed pictures of your child's lungs. This test can show the location, extent, and severity of lung disease.
• Lung function tests. These tests measure how much air your child can breathe in and out, how fast he or she can breathe air out, and how well your child's lungs deliver oxygen to the blood. Lung function tests can assess the severity of lung disease. Infants and young children may need to have these tests at a center that has special equipment for children.
• Bronchoalveolar lavage (BRONG-ko-al-VE-o-lar lah-VAHZH). For this procedure, the doctor injects a small amount of saline (salt water) through a tube inserted in the child's lungs. The fluid helps bring up cells from the tissues around the air sacs. The doctor can then look at these cells under a microscope. This procedure can help detect an infection, lung injury, bleeding, aspiration, or an airway problem.
• Various tests to rule out conditions such as asthma, cystic fibrosis, acid reflux, heart disease, neuromuscular disease, and immune deficiency.
• Various tests for systemic diseases linked to chILD. Systemic diseases are diseases that involve many of the body's organs.
• Blood tests to check for inherited (genetic) diseases and disorders.

If these tests don't provide enough information, your child's doctor may recommend a lung biopsy. A lung biopsy is the most reliable way to diagnose chILD and the specific disease involved.

• A lung biopsy is a surgical procedure that's done in a hospital. Before the biopsy, your child will receive medicine to make him or her sleep.
• During the biopsy, the doctor will take small samples of lung tissue from several places in your child's lungs. This often is done using video-assisted thoracoscopy (thor-ah-KOS-ko-pe).
• For this procedure, the doctor inserts a small tube with a light and camera (endoscope) into your child's chest through small cuts between the ribs. The endoscope provides a video image of the lungs and allows the doctor to collect tissue samples.
• After the biopsy, the doctor will look at these samples under a microscope.

Treatment

Childhood interstitial lung disease (chILD) is rare, and little research has been done on how to treat it. At this time, chILD has no cure. However, some children who have certain diseases, such as neuroendocrine cell hyperplasia of infancy, may slowly improve over time. Current treatment approaches include supportive therapy, medicines, and, in the most serious cases, lung transplants.

Supportive Therapy

• Supportive therapy refers to treatments that help relieve symptoms or improve quality of life. Supportive approaches used to relieve common chILD symptoms include:
• Oxygen therapy. If your child's blood oxygen level is low, he or she may need oxygen therapy. This treatment can improve breathing, support growth, and reduce strain on the heart.
• Bronchodilators. These medications relax the muscles around your child’s airways, which helps open the airways and makes breathing easier.
• Breathing devices. Children who have severe disease may need ventilators or other devices to help them breathe easier.
• Extra nutrition. This treatment can help improve your child's growth and help him or her gain weight. Close monitoring of growth is especially important.
• Techniques and devices to help relieve lung congestion. These may include chest physical therapy (CPT) or wearing a vest that helps move mucus (a sticky substance) to the upper airways so it can be coughed up. CPT may involve pounding the chest and back over and over with your hands or a device to loosen mucus in the lungs so that your child can cough it up.
• Supervised pulmonary rehabilitation (PR). PR is a broad program that helps improve the well-being of people who have chronic (ongoing) breathing problems.

Medicines

• Corticosteroids are a common treatment for many children who have chILD. These medicines help reduce lung inflammation.
• Other medicines can help treat specific types or causes of chILD. For example, antimicrobial medicines can treat a lung infection. Acid-blocking medicines can prevent acid reflux, which can lead to aspiration.

Lung Transplant

• A lung transplant may be an option for children who have severe chILD if other treatments haven't worked.
• Currently, lung transplants are the only effective treatment for some types of chILD that have a high risk of death, such as alveolar capillary dysplasia and certain surfactant dysfunction mutations.
• Early diagnosis of these diseases gives children the chance to receive lung transplants. So far, chILD doesn't appear to come back in patients' transplanted lungs.

NIH genetic and rare disease info

• NIH info on Children's interstitial lung disease

Children's interstitial lung disease is a rare disease.

Children's interstitial lung disease Resources
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