Interstitial lung disease (ILD)

Interstitial lung disease (ILD)

Overview

Interstitial lung disease (ILD) is a group of disorders in which the lungs develop significant and sometimes disabling inflammation, scar tissue or fibrosis within the tissues. This may be due to pulmonary fibrosis or other similar problems. Idiopathic pulmonary fibrosis (IPF) is the most common ILD. Other names for ILD are:

  • Diffuse parenchymal lung disease
  • Alveolitis
  • Idiopathic pulmonary pneumonitis (IPP)

ILD limits the transfer of oxygen across the membranes of the lung, making breathing difficult. Around the air sacs in the lungs is tissue called the interstitium. When the tissue is stiff or scarred, the body experiences a lack of oxygen because the air sacs aren’t able to fully expand.

Causes

ILD has many causes, and sometimes may occur for no reason (idiopathic ILD). According to the U.S. National Library of Medicine at the National Institutes of Health, causes include:

  • Autoimmune diseases such as lupus, rheumatoid arthritis, sarcoidosis and scleroderma
  • Lung inflammation due to breathing in a foreign substance, including particular types of  dust, fungus or mold (hypersensitivity pneumonitis)
  • Medications such as nitrofurantoin, sulfonamides, bleomycin, amiodarone, methotrexate, gold salts, infliximab and etanercept
  • Radiation treatment of the chest
  • Occupational lung disease (working with or around asbestos, coal dust, cotton dust and silica dust, in jobs such as coal mining, sand blasting or working on a ship)
  • Cigarette smoking, which may increase the risk of developing some form of ILD and may cause the disease to be more severe.

Symptoms

  • Shortness of breath, the main symptom of ILD, causing you to breathe faster, harder, or more shallowly, or to take more deep breaths
    • Initially, this symptom may not be severe and occurs with exercise, stair climbing and other physically demanding activities.
    • Over time, it may become more obvious during less strenuous activity, such as ordinary daily tasks, and then even when you are eating or talking. To take deep breaths, you may need to lean forward.
  • A dry cough, a symptom most people with ILD have
    • Gradually, weight loss, fatigue and muscle and joint pain occur.
  • “Clubbing” as the disease progresses: a condition in which there may be abnormal enlargement of the base of the fingernails
  • Cyanosis, in which your lips, skin or fingernails turn blue because of inadequate blood oxygen.
  • Frequent headaches
  • Sleepiness or confusion
  • Fever
  • Coughing up dark mucus

Diagnosis

A routine physical exam with a stethoscope can detect dry, crackling sounds in the chest. People who are heavily exposed to known causes of lung disease in the workplace are usually routinely screened for lung disease. According to the NLM/NIH, other diagnostic tests that may be performed are:

  • Blood tests to check for connective tissue diseases
  • Bronchoscopy, with or without biopsy
  • Chest X-ray
  • Computed tomography (CT) scan of the chest
  • Echocardiogram 
  • Open lung biopsy
  • Measurement of the blood oxygen level at rest or when active
  • Pulmonary function tests
  • Six-minute walk test (to see how far you can walk in six minutes and how many times you need to stop to catch your breath)

Treatment

  • If an autoimmune disease is causing the problem, drugs may be prescribed to suppress the immune system and reduce swelling in the lungs.
  • For those with advanced ILD, a lung transplant may be the only effective treatment.
  • If the problem has produced low blood oxygen levels, patients can receive home-based oxygen therapy, after instruction by a respiratory therapist.

Support

If there is no applicable medical or surgical treatment for your particular type of ILD, there are measures you can take toward lung rehabilitation:

  • Quit smoking
  • Learn different breathing methods
  • Arrange your home to help you save your energy when moving around
  • Eat the proper amount of calories and nutrients
  • Stay active and increase your strength

(855) 432-7811

Make an appointment

Thoracic surgery

Experts in minimally invasive procedures, Northwell Health has one of the leading robotic thoracic surgical programs in the United States.