Diagnosis of Interstitial Lung Disease (ILD)

Abnormalities on a chest x-ray may be the first evidence of ILD, but they are seldom specific to a particular disorder. For example, lung function tests typically show a decrease in total lung capacity, the amount of air in the lungs after a deep inhalation. Another common abnormality is a decrease in diffusing capacity, a measurement of how well gas passes across the membranes in the lungs. Again, these deviations do not distinguish among the various underlying causes of ILD.

The results from high-resolution chest computed tomography (CT) scans may be helpful in individuals suspected of having ILD. For example, a scan showing changes in which the edge of a severely damaged lung resembles a honeycomb may eliminate the need for a lung biopsy, because it is so specific for one form of ILD.

In some cases, it is possible to make a definitive diagnosis based on examination of lung fluids obtained using bronchoalveolar lavage (washing out the lungs by passing a saline solution into them through the thin tube of a bronchoscope inserted into the mouth or nose). A lung biopsy may be necessary to make a definitive diagnosis and determine the severity of the disease. The least invasive procedure is a biopsy performed through a bronchoscope.

This procedure can be performed on an outpatient basis, however, the small size of the tissue sample may not be sufficient for certain diagnoses. If this approach is inconclusive, an open surgical biopsy in a hospital would be necessary.

ILD Prevention

Avoiding potential triggers of ILD is crucial. Wearing appropriate masks and monitoring exposure to irritants in certain work environments (for example, mining and jobs that involve working with asbestos or metal dusts) are important precautions to take.

Treatment for Interstitial Lung Disease

Several types of ILD, most notably sarcoidosis and hypersensitivity reactions, get better on their own, or they respond to treatment with corticosteroids. Avoidance of toxin or allergen exposures also can be beneficial.

Unfortunately, for some other causes of ILD, there is no specific treatment to slow the progression of the disease or reverse the damage that has already occurred. Further inflammation and scarring of lung tissue weaken lung function and can lead to right heart failure, which slows the movement of blood through the heart.

Although corticosteroid treatment can improve pulmonary sarcoidosis, it often fails to relieve symptoms in people who have other forms of ILD. Corticosteroid treatment is generally combined with chemotherapy drugs such as cyclophosphamide (Cytoxan) or azathioprine (Imuran) for patients with IPF in a limited-time trial, but this treatment is often not effective for this debilitating and progressive condition.

Recently, the search for new idiopathic pulmonary fibrosis (IPF) treatments has received a boost through the National Heart, Lung, and Blood Institute's (NHLBI's) Idiopathic Pulmonary Fibrosis Network (IPFnet), a group of 12 research centers dedicated to the study of IPF. These centers are developing and conducting clinical trials that are designed to identify better therapies for IPF in both its early and advanced stages.

In October 2014, the U.S. Food and Drug Administration (FDA) approved two new medications—pirfenidone (Esbriet) and nintedanib (Ofev)—to treat IPF. Esbriet should not be used in people with severe liver problems, end-stage kidney disease, or those who are on dialysis. Ofev should not be used in people with moderate to severe liver problems and can cause serious birth defects in women who are pregnant (women should not become pregnant for at least 3 months after taking the drug).

Esbriet should be taken with food to reduce side effects and can cause the following:

  • Abdominal pain
  • Diarrhea
  • Dizziness
  • Fatigue
  • GERD (gastroesophageal reflux disease)
  • Headache
  • Increased sensitivity to the sun
  • Insomnia
  • Loss of appetite
  • Muscle pain
  • Respiratory infection
  • Sinus infection
  • Vomiting
  • Weight loss

Ofev can cause:

  • Abdominal pain
  • Diarrhea
  • Headache
  • High blood pressure (hypertension)
  • Increased liver enzymes
  • Loss of appetite
  • Nausea
  • Weight loss

Updated by Remedy Health Media

Publication Review By: Peter B. Terry, M.D., M.A.

Published: 19 Aug 2011

Last Modified: 16 Oct 2014