Interstitial Lung Disease | Diagnosis & Treatments

At Boston Children’s Hospital, we know that getting an accurate diagnosis is the first step to effectively treating your child with interstitial lung disease (ILD).

How is interstitial lung disease diagnosed?

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

What tests are used to diagnose ILD?

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

Treatments for interstitial lung disease in children

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 shelp 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.