Interstitial Lung Diseases

What Are Interstitial Lung Diseases (ILD)?

Interstitial lung diseases are a group of disorders that result from damage to the "interstitium," the area at the end of the airways where gas exchange occurs, which contains air sacs, blood vessels, and various cells. It is also known as lung thickening or hardening.

How Is Interstitial Lung Disease Diagnosed?

The diagnosis of interstitial lung diseases is made through a detailed evaluation of the patient's symptoms, detection of abnormal lung sounds during examination, demonstration of reduced lung capacity and impaired gas exchange in pulmonary function tests, and identification of abnormal appearances in high-resolution lung tomography.

Diagnosing ILD can be challenging. It requires a thorough examination of all the patient's signs and symptoms together. Unfortunately, there is no single method for making the diagnosis.

Groups of Interstitial Lung Diseases

Interstitial lung diseases are classified into two groups: those with a known cause and those with an unknown cause.

In the group with a known cause:

• Drug-induced ILD (immunosuppressive drugs used in chemotherapy and transplant patients)
• Radiation-induced ILD
• ILD associated with rheumatologic diseases (rheumatoid arthritis, scleroderma, Sjögren's disease)
• Asbestos and silica exposure
• Smoking-related ILD
• ILD due to inhalation of various dusts and allergens (hypersensitivity pneumonitis)

In the group with an unknown cause:

• Idiopathic pulmonary fibrosis (IPF)
• Cryptogenic organizing pneumonia
• Nonspecific interstitial pneumonia
• Lymphocytic interstitial pneumonia
• Acute interstitial pneumonia
• Sarcoidosis
• Pulmonary Langerhans cell histiocytosis
• Lymphangioleiomyomatosis

What Causes Interstitial Lung Disease?

Interstitial lung diseases are associated with smoking, gastroesophageal reflux, various medications, radiation therapy, and various rheumatic diseases. However, often no cause is found. In rare cases, it can be inherited.

What Are the Symptoms of Interstitial Lung Disease?

The most common symptoms of interstitial lung disease are a dry cough lasting at least 3 months and shortness of breath.

Is Interstitial Lung Disease a Cancer?

Interstitial lung disease is not a cancer. It is not contagious.

Is Interstitial Lung Disease Fatal?

Some interstitial lung diseases can be fatal. In cases where the disease is not diagnosed for a long time and progresses, severe respiratory failure can occur.

Late Stages of Interstitial Lung Disease

In the late stages of interstitial lung disease, respiratory failure occurs due to impaired gas exchange in the lungs. These patients should be referred to relevant centers early for lung transplantation.

Treatment of Interstitial Lung Disease

The treatment of interstitial lung disease usually involves treating the underlying cause, often with corticosteroids. In idiopathic pulmonary fibrosis, medications that stop lung thickening and hardening are used.

To summarize the treatment generally:

Drug-induced ILD: Discontinuation of the drug and corticosteroid treatment

Radiation-induced ILD: Corticosteroid treatment

ILD associated with rheumatoid diseases: Treatment of the underlying rheumatic condition

Smoking-related ILD: Quitting smoking and, if necessary, corticosteroid treatment

ILD due to inhalation of various dusts and allergens (hypersensitivity pneumonitis): Avoidance of allergens and corticosteroid treatment

Idiopathic pulmonary fibrosis (IPF): Two drugs are used to stop lung hardening and prevent loss of function (Pirfenidone or Nintedanib)

Cryptogenic organizing pneumonia: Corticosteroids

Nonspecific interstitial pneumonia: Corticosteroids

Acute interstitial pneumonia: It is a severe condition. High-dose corticosteroids are started, and the patient may need to be placed on a ventilator.

Sarcoidosis: Treated with corticosteroids in some cases, but often resolves on its own.

What Should Be Done for Interstitial Lung Disease?

If you have a dry cough and/or shortness of breath lasting more than 3 months and worsening over time, you should definitely see a pulmonologist.

If you have a rheumatologic disease, you should regularly visit a pulmonologist. Quit smoking. If you have gastroesophageal reflux, get it treated. If you are diagnosed with interstitial lung disease, take it seriously, take your medications, and attend your doctor appointments.

Interstitial lung disease is not cancer, so don't be afraid!

Thickening of the lung tissue is usually an irreversible problem. Although the treatments applied during this process may slow the progression of the disease, it may not always be completely stopped. At this point, lung transplantation should be considered.

When interstitial lung disease is detected early, it can be treated.