Interstitial lung disease Symptoms & Causes


What you need to know about interstitial lung disease

Interstitial lung disease (ILD, chILD) isn’t actually a single disease—rather, it’s a group of rare lung diseases in which the cells and tissues of the lungs are damaged and don’t work the way they should. There are various levels of severity, treatments and outcomes.

The ILD category of diseases includes:

  • 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 causes of ILD differ based on the type.

  • Some have a cause we know and understand, but others are less well understood.
  • Some babies are born with ILD, and some types of ILD (e.g., surfactant problems) 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.
  • Some are caused by another disease, and some are even caused by treatments for other medical problems.

National and local research is underway to understand more about ILD, including studies with the participation of Boston Children’s clinicians. To learn more, visit our Research & Clinical Trials page

Signs and symptoms 

Even though there are many diseases in the ILD 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 more than a few of these symptoms may benefit from an evaluation for ILD by pediatric pulmonary specialist, who may recommend that your child have further testing to determine your child’s condition.