Interstitial Lung Disease

Also known as: ILD, IPF, cryptogenic fibrosing alveolitis, CFA.

What is interstitial lung disease?

Childhood Interstitial lung disease is a broad term that applies to a rare complex group of different conditions in children (mostly infants) that primarily share a common abnormality of the air sacs and the scar like tissue that surrounds them, leading to difficulty in getting oxygen to the tissues and removing the carbon dioxide produced by them during metabolism. In children the microscopic picture of lung tissue in ILD differs from that of the same disease in adults and the diagnosis is not always easy to make as the clinical presentation may not reflect the microscopic picture.

What causes interstitial lung disease?

Interstitial lung disease occurs when lung tissue is damaged and the healing process results in inflammatory scar like tissue being laid down, in a child’s lung which is still developing. Some forms have a family history, while with others exposure to infections (viral and others), allergy, organic dusts, hazardous fumes or chemicals, medication, radiation, certain other systemic diseases and many other disorders may play a role. Often no cause can be found.

What are the symptoms of interstitial lung disease?

Symptoms vary by age; sometimes symptoms are so insidious that no one notices anything wrong. Others, especially in the newborn and infant,  present with rapid breathing, shortness of breath, difficulty feeding with cyanosis (a bluish color to the skin, tongue and nail beds) a dry, persistent cough, and failure to gain weight. Older children may complain of chest pain, cough up blood, or wheeze.

What are interstitial lung disease care options?

Because of the multiple disorders that can give rise to ILD, treatments depend on the underlying cause. General principles of care include attention to nutrition and growth, immunizations and treatment of secondary infections. Other treatments include bronchodilators, oxygen therapy, and medications which attempt to diminish the inflammation that occurs. Lung biopsy may be required for diagnosis and potentially lung transplantation may be considered.

Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: September 09, 2019 03:40 PM