Interstitial Lung Disease (Pediatric)

Children’s interstitial lung disease, also called diffuse lung disease, refers to a heterogeneous group of rare respiratory disorders diffusely affecting the small airways and/or walls of the alveolar sacs of the lung where gas exchange occurs

Each type of interstitial lung disease is different, but they all limit the body’s ability to get the oxygen it needs. While some types of interstitial lung disease can be managed and get better over time, many are progressive and can cause permanent scarring of the lung tissue.

The University of Michigan C.S. Mott Children’s Hospital provides comprehensive care for children with interstitial lung disease. Our pediatric pulmonology team, the largest in Michigan, has special expertise in diagnosing and treating the full range of childhood interstitial lung diseases. We are one of a small number of centers participating in the Children’s Interstitial Lung Disease (ChILD) research network.

Diagnosing interstitial lung disease

Interstitial lung disease can be difficult to diagnose, but accurate and early diagnosis is fundamental to effective treatment and management of these diseases. It’s important to choose a center with the diagnostic experience and expertise needed to identify specific respiratory diseases.

Our state-of-the-art pediatric pulmonary clinic offers advanced diagnostic tests including:

  • Advanced CT scan capabilities, including controlled ventilation chest CT
  • Infant pulmonary function testing
  • Bronchoscopy and bronchoalveolar lavage
  • Pediatric genetic analysis
  • Lung biopsies, when needed

Interstitial lung diseases in children are very rare, and can fall into a range of categories, including:

  • Infant lung disorders related to lung development, such as alveolar capillary dysplasia, or abnormal lung growth (pulmonary hypoplasia, bronchopulmonary dysplasia),
  • Lung disorders related to surfactant function or metabolism, such as surfactant protein deficiencies, ABCA3 deficiency and pulmonary alveolar proteinosis
  • Neuroendocrine hyperplasia of infancy, pulmonary interstitial glycogenosis
  • Lung disorders related to the body’s response to infection, drugs and environmental toxins (hypersensitivity pneumonitis, bronchiolitis obliterans)
  • Aspiration syndromes
  • Disorders related to systemic diseases such as connective tissue diseases, sarcoidosis, storage diseases and Langerhan cell histiocytosis
  • Lung disorders associated with a weakened immune system, including opportunistic infections after solid organ and bone marrow transplantation
  • Lung disorders related to transplant rejection

There are also many disorders that resemble interstitial lung disease. Our team has the experience needed to accurately identify these disorders, and treat them accordingly.

Treating interstitial lung disease

Our skilled pediatric pulmonology team will work with your family to individualize your child’s therapy based on his or her specific condition. Treatments will vary based on specific diagnosis and your child’s individual medical history, and can include:

  • Oxygen therapy, and mechanical ventilation if required
  • Nutrition services
  • Anti-inflammatory drugs such as certain antibiotics, systemic corticosteroids (including pulse therapy), hydroxychloroquine
  • Therapies to clear mucus from the lungs, such as hypertonic saline, oscillatory positive expiratory pressure therapy or high frequency chest wall oscillation
  • Intravenous immunoglobulin therapy

Choosing an interstitial lung disease treatment program

Since interstitial lung diseases are so rare, there are very few hospitals in the country that have the tools and experience necessary to diagnose and treat these complex conditions.  

Led by the largest pediatric pulmonology team in Michigan, we collaborate with pediatric specialists from across the University of Michigan in order to provide the most comprehensive, coordinated care for children and families living with interstitial and diffuse lung diseases.

In addition, as part of the ChILD Foundation Research Network, we work with other pediatric pulmonary centers across the country to advance research and clinical trials that can lead to better care for children with ILD. 

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To schedule an appointment with our interstitial lung disease team, please call us at 734-764-4123.