Interstitial Lung Diseases


photo of bare chested man  with a lung graphic

What are interstitial lung diseases?

Interstitial lung diseases comprise more than 200 lung disorders that primarily affect the tissue around the air sacs, called the interstitium. These diseases – many of them rare – lead to respiratory symptoms similar to asthma or COPD, such as coughing, wheezing, chest tightness and shortness of breath. They also decrease lung volume and function, and cause inflammation and scarring that make it difficult to breathe.

What is chronic eosinophilic pneumonia?

Eosinophils are a type of white blood cells that form in response to allergens or infections and help fight disease, particularly respiratory conditions. When eosinophils amass in the lungs and the bloodstream, it can lead to chronic eosinophilic pneumonia.

What causes chronic eosinophilic pneumonia?

What causes chronic eosinophilic pneumonia is unknown. Some patients have pre-existing severe asthma or a history of allergies. The disease occurs in more women than men, usually around middle age, although some children do develop it. 

What should I know about chronic eosinophilic pneumonia?

  • Symptoms include shortness of breath, cough, fatigue, night sweats, low grade fevers, and unintended weight loss.
  • Oral corticosteroids are the primary treatment. 
  • Relapse is common, especially if treatment is stopped within 6 months. Some patients also develop severe asthma post-treatment.

What is Churg-Strauss Syndrome (eosinophilic granulomatosis with polyangiitis (EGPA)?

Churg-Strauss syndrome, or eosinophilic granulomatosis with polyangiitis (EGPA), is a form of vasculitis – a family of diseases characterized by inflammation of the blood vessels, which can restrict blood flow and damage vital organs and tissues. EGPA is one of the rarest forms of vasculitis and primarily affects small blood vessels. 

EGPA can affect the lungs, sinuses, skin, heart, intestinal tract, kidneys, nerves and other organs. The disease involves a blood protein (anti-neutrophil cytoplasmic antibody) that attacks the body’s own cells and tissues.

Individuals with EGPA usually have a history of asthma or allergies. EGPA can occur at any age, however the average age of diagnosis is between 35 and 50 years old. Women and men are affected equally.

What should I know about EGPA?

  • Almost all EGPA patients have asthma and/or sinus polyps, and a higher-than-normal level of eosinophils.
  • Symptoms can range from mild to life-threatening, depending on which organs are involved and the extent of disease. Symptoms may include fever, fatigue, rapid and sudden weight loss, muscle and joint pain, skin rashes, numbness or tingling of the hands or feet, increasing shortness of breath or coughing and abdominal pain.
  • EGPA is serious but treatable. Treatment typically includes oral corticosteroids used in combination with medications that suppress the immune system.
  • In late 2017, a new medication was added to the treatment toolbox. FDA approved the use of the biologic drug mepolizumab for the treatment of EGPA in adults.
  • Even with effective treatment, EGPA is a chronic illness with cycles of relapse and remission that can cause serious health problems, so ongoing medical care is necessary.

What is lymphangioleiomyomatosis (LAM)?

Lymphangioleiomyomatosis is a rare lung disease that occurs almost exclusively in women. It results from an abnormal growth of smooth muscle cells in the lungs, lymphatics system and kidneys. This causes cysts to develop and leads to loss of lung function, fluid in the chest and tumors on kidneys. Sometimes the cysts rupture, allowing air into the chest cavity, causing extreme discomfort.

What should I know about LAM?

  • The most common symptoms are coughing, wheezing, chest tightness and shortness of breath. Other symptoms include blood in urine, enlarged lymph nodes and swelling on the body, usually in the abdomen or feet.
  • LAM worsens over time, but its progression is usually slow. There is no cure, however medications are available to help improve lung function and make breathing easier.
  • If the level of oxygen in the blood is low, doctors may suggest oxygen therapy given through nasal prongs or a mask. For those with advanced disease, a lung transplant may be considered – but again, this is not a cure.

What is idiopathic pulmonary fibrosis (IPF)?

When scar tissue forms in the body, the medical term is “fibrosis” – and when scarring happens in the lungs, it’s pulmonary fibrosis. Many cases of pulmonary fibrosis are idiopathic, meaning the cause is unknown.

IPF is a very serious condition that is usually found in adults middle-aged and older. The lungs aren’t able to transport oxygen and eventually, the patient can no longer breathe. In addition to scarring, some experience thickening tissue in the lungs and this makes it hard for the lungs to work.

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    What should I know about IPF? 

    • Every person diagnosed with pulmonary fibrosis has a unique experience with the disease. Some patients remain in stable condition for extended periods of time; others may experience a rapid progression of symptoms; while another group may experience a stepwise deterioration over time. 
    • Symptoms include shortness of breath, dry cough, fatigue, unexplained weight loss, aching muscles and joints as well as clubbing of the fingers or toes.
    • There is no cure for IPF.  The average life expectancy of someone with pulmonary fibrosis is 3-5 years but if it’s caught early, treatment can help slow the progression of the disease.
    • Treatment is highly personalized, based upon a patient’s medical history and other conditions. Medications are available to help slow the progression of the disease. Oxygen therapy and pulmonary rehabilitation may also help. For the most severe cases, a lung transplant may be considered.

    What is pneumoconiosis? 

    If you breathe in dust particles at work – perhaps you’re in construction or you work in a coal mine, or you’re exposed to asbestos in an office building, silica at a rock and sand foundry or cotton dust from textiles – then you’re at risk for pneumoconiosis, a progressive lung disease. 

    Sometimes called occupational lung disease, pneumoconiosis occurs when these harmful particles reach the lungs, causing an immune system response. This can lead to severe inflammation and scarring (fibrosis).

    What should I know about pneumoconiosis?

    • Pneumoconiosis can take years to develop. Symptoms are similar to asthma and other respiratory diseases: shortness of breath; coughing with phlegm; and chest tightness.
    • There is no cure for pneumoconiosis – treatment focuses on managing the disease. Patients may be prescribed quick-relief and long-acting controller inhalers, as well as undergo oxygen therapy to improve lung function.
    • Patients should work with doctors and employers to ensure the workplace has proper health and safety measures in place, including wearing protective clothing and masks. Lifestyle changes such as maintaining a healthy weight, quitting smoking (if you smoke) and frequent handwashing may also help.

    What is sarcoidosis?

    Sarcoidosis occurs when groups of immune cells form lumps (called granulomas) on various organs in the body, usually the lungs and lymph nodes in the chest. While no one knows what causes sarcoidosis, it is believed that inflammation and an overactive immune system may play a role.Sarcoidosis can cause breathing problems, reduced lung function and sometimes permanent lung damage (including scarring). The disease can affect anyone, but people over 20 years of age and women are more likely to develop it. African-Americans are at higher risk for sarcoidosis than Caucasians and may experience more severe symptoms.

    What should I know about sarcoidosis?

    • Some people with sarcoidosis never experience symptoms and/or never require treatment.
    • People with sarcoidosis may experience fever, fatigue and other symptoms depending on the organ that is affected: coughing or wheezing; shortness of breath; chest pain; skin rashes or hives; joint pain or stiffness; eye irritation or dryness; fast heart rate or lightheadedness; night sweats.
    • Determining whether treatment is needed and what type depends on the symptoms. Oral corticosteroids may be prescribed.

    How are interstitial lung diseases diagnosed?

    Doctors will perform a physical exam and evaluate your family medical history. They may also seek to rule out diseases that have similar symptoms.

    Diagnostic tests that search for inflammation and/or scarring in the lungs may include:

    • Lung function testing
    • Blood tests
    • Chest X-ray
    • CT scan
    • Lung tissue biopsy
    • Bronchoscopy to examine the lungs or get a tissue sample

    What are some other interstitial lung diseases?

    • Bronchiolitis obliterans-organizing pneumonia (BOOP)
    • Chronic pulmonary edema
    • Drug-induced pulmonary injury
    • Goodpasture syndrome
    • Histiocytosis X
    • Hypersensitivity pneumonitis
    • Idiopathic pulmonary hemosiderosis

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    What are the side effects of oral corticosteroids?

    Short-course and long-term use of oral corticosteroids (such as prednisone) to treat interstitial lung diseases may come with side effects, some of them serious. Talk with your doctor about these side effects if OCS medications are prescribed.

    Short-course use:

    • Eye problems
    • Weight gain, particularly in abdomen, face
      and neck
    • Swelling in lower legs
    • High blood pressure
    • Muscle weakness
    • Mood swings and aggressive behavior
    • Depression and anxiety
    • Sleep problems


    Long-term use:

    • Slow growth in children
    • Diabetes
    • Increased risk of infections
    • Bone weakness
    • Easy bruising of the skin
    • Reduced hormone production