Interstitial Lung Disease

Boston Children’s Hospital specializes in treating pediatric respiratory diseases, including interstitial lung disease (ILD or chILD).

Interstitial lung disease is not a single disease but rather, is a group of rare lung diseases that range from mild to severe and can make it difficult to breathe. This is because these lung diseases can interfere with the exchange of oxygen and carbon dioxide and can cause fluid and other materials to collect in the lungs.

Different types of interstitial lung diseases require different forms of treatments and can have different outcomes.

The types of interstitial lung diseases we treat include:

• Acute interstitial pneumonia/pneumonitis
• Alveolar capillary dysplasia
• Alveolar hemorrhage syndromes (pulmonary capillaritis, acute idiopathic pulmonary hemorrhage of infancy and idiopathic pulmonary hemosiderosis)
• Aspiration-associated lung disease
• Autoimmune- or rheumatologic-related lung disease
• Bone marrow transplant-associated lung disease/graft-versus-host lung disease
• Bronchiolitis obliterans
• Cryptogenic organizing pneumonia (formerly known as bronchiolitis obliterans organizing pneumonia or BOOP)
• Desquamative interstitial pneumonia
• DNA repair disorders
• Drug-induced lung disease
• Eosinophilic pneumonias
• Follicular bronchiolitis
• Growth abnormalities
• Hypersensitivity pneumonitis
• Immune-mediated lung disease
• Immunocompromised host associated lung disease
• Lymphocytic interstitial pneumonia
• Lysosomal storage disorders
• Neuroendocrine hyperplasia of infancy
• Nonspecific interstitial pneumonia
• Pulmonary alveolar proteinosis
• Pulmonary interstitial glycogenosis
• Pulmonary alveolar microlithiasis
• Pulmonary histiocytosis
• Pulmonary lymphagiectasia
• Pulmonary lymphangiomatosis
• Pulmonary vascular disorders
• Pulmonary sarcoidosis
• Radiation-induced lung disease
• Surfactant gene mutation-associated lung disease (SP-B, SP-C, ABCA3, TTF-1, GM-CSF receptor)

The cause of interstitial lung disease can be different for different children and can vary by type. For instance:

• Some babies are born with ILD, and some types of ILD are inherited. But many children don’t become affected with ILD until later in childhood.
• For some children, ILD will show up after a “trigger” event, such as a cold or viral infection.
• ILD can be caused by another disease, or by treatments for other medical problems.

Even though there are many diseases in the interstitial lung disease category, a lot of them share certain signs and symptoms. Not every child will have every symptom, but symptoms could include:

• Rapid breathing, difficulty breathing, or shortness of breath
• Using rib or neck muscles to help breathe
• Failure to thrive: Failure to gain weight and/or height
• Crackles, wheezing, or other abnormal sounds in lungs
• Repeated occurrences of pneumonia, bronchitis, and/or cough

Children with multiple symptoms may benefit from an evaluation for ILD by one of our pediatric pulmonary specialists.

Before establishing a diagnosis of ILD, doctors must rule out other possible causes of the symptoms. This is called making a differential diagnosis. Some of the other conditions that cause similar symptoms include:

• Cystic fibrosis
• Acid reflux (gastoresophageal reflux disease)
• Heart problems
• Aspiration
• Scoliosis or other neuromuscular disease
• Abnormalities of the chest wall
• Immune deficiency

There isn’t one specific test that can cover the many these types of lung diseases. Common tests to help diagnose interstitial lung disease include:

• Chest X-ray and high-resolution computed tomography (HRCT): Takes images of the lungs
• Pulse oximetry: Measures how well the blood is carrying oxygen
• Pulmonary function tests (PFTs): Series of breathing tests that determine how much, and how well, the lungs take in and expel (let out) air
• Exercise testing: Determines exercise tolerance and oxygen levels during exercise
• Bronchoscopy with bronchoalveolar lavage: Examines the airways and looks for infection and inflammation in the lungs
• Lung biopsy: Current “gold standard” for diagnosing and categorizing ILD
• Genetic tests: Test blood and other tissue to detect gene disorders (very few ILD diseases have known genetic causes)
• Barium swallow: Detects signs of aspiration (breathing foreign objects into airways) while swallowing food and liquids of different consistencies
• Ultrasound (echocardiography): Evaluates heart function
• Sweat testing: Testing to exclude cystic fibrosis
• Skin testing: Testing to exclude tuberculosis
• Electrocardiogram (EKG) and/or cardiac catheterization: Detects heart defects or pulmonary hypertension
• pH probe: Detects acid reflux
• Other lab tests: Look at urine, mucus, blood, and feces

There is no single treatment for interstitial lung disease. Our clinicians will develop a personalized treatment plan based on each child’s specific diagnosis and symptoms. We have a multi-specialty group that meets on a frequent basis to review evaluation and treatment plans.

ILD treatment goals

The goals of interstitial lung disease treatment include:

1. Preventing low oxygen levels: Low oxygen levels are detrimental to growth and development and can strain the heart, so we pay close attention to diagnosing and treating low blood oxygen levels and provide oxygen as needed. Occasionally other devices (non-invasive ventilation/pressure machines or tracheostomy tubes/ventilators) are needed to maintain gas exchange.
2. Reducing the excess work of breathing: It is important to reduce the work required to breathe so that your child can use more of the calories required to grow. We often use anti-inflammatory therapies (such as steroids or immune modulation medications) to treat lung inflammation, which can make it harder to breathe. We also do things like chest PT to help remove built up mucous in the airways that can cause a lack of oxygen.
3. Providing nutritional support as needed to promote growth: Children with ILD may work harder to breathe and may find it difficult to take in enough calories to supply the energy to breathe and grow. We have a nutritionist on our team who provides expert advice on how to add supplemental calories and needed nutrients. Occasionally our ILD patients require therapy with additional calories provided through feedings tubes; if we think your child might need this, we will discuss this with you before moving forward.
4. Preventing further damage to the lungs from the underlying condition and associated conditions: Infections can cause a flare-up in respiratory symptoms in children with lung diseases, so we recommend that you keep your child's vaccinations up to date. We'll also help you keep your child's airways clear using clearance therapy to prevent mucous building up. This will help reduce the risk of infections. If an infection occurs, we will treat it aggressively. Some specific ILDs are associated with problems in other organ systems. Through our multi-disciplinary group, we work to coordinate and consolidate care of associated conditions. Sometimes lung transplantation becomes a treatment option for children with ILD. If this is the case for your child, the Lung Transplant Program team here at Boston Children's Hospital will provide expert review and opinion, as well as other services and support.

Our Interstitial Lung Disease Program consists of doctors, nurses, nutritionists, and social workers who coordinate to provide a full diagnosis and evaluation of each child with ILD.

We work with families to develop an individualized treatment plan based on the child’s specific diagnosis. We also help families arrange home health care support and supplies, transportation services, and visiting nursing services.

If needed, our Division of Pulmonary Medicine provides expert review and opinion, as well as other services and support for lung transplantation.

The clinicians in the Division of Pulmonary Medicine are committed to advancing the understanding of interstitial lung disease and other pulmonary diseases (lung diseases) through a variety of research efforts.

Our ILD program is a part of the Children's Interstitial Lung Disease Research Network (ChILDRN), which is a group of physician scientists from across the country who work together to conduct research and promote awareness of these rare disorders. ChILDRN also developed the pediatric interstitial/diffuse lung disease patient registry and works closely with the Children's Interstitial Lung Disease (ChILD) Foundation.