Childhood Interstitial Lung Disease

The interstitium in the lung is the tissue that lies between the alveoli in the lungs. In normal lungs, this tissue is very thin to allow oxygen and carbon dioxide to cross between the surface of the alveoli (air sacs) and the capillaries that carry blood. If this tissue becomes thickened or scarred, it makes it harder to absorb oxygen into the blood and to release carbon dioxide when the person exhales, which can make the patient quite sick. Interstitial pneumonia is inflammation of the interstitial tissue (not the alveoli themselves).

Symptoms

In the beginning, these patients can have a dry, unproductive cough, shortness of breath, and/or a low grade fever. Unfortunately, this form of interstitial lung disease is rapidly progressing and is often fatal.

The common diagnostic tests are chest x-ray, chest CT scan, and lung biopsy. High resolution CT scans show patchy or geographic ground glass opacity initially, followed by architectural distortion (honeycomb appearance with small cystic spaces surrounded by fibrosis), and traction bronchiectasis (dilated airways surrounded by fibrosis). If biopsied, the histology shows diffuse alveolar damage.

Corticosteroids and other treatments are often ineffective and many patients require mechanical ventilation and intensive care support. Even with medical intervention, this is a serious form of chILD and can be fatal.