Interstitial lung disease
Interstitial lung disease is a group of lung disorders in which the lung
tissues become inflamed and then damaged.
Causes
The lungs contain tiny air sacs (alveoli), which is where oxygen is
absorbed. These air sacs expand with each breath.
The tissue around these air sacs is called the interstitium. In people
with interstitial lung disease, this tissue becomes stiff or scarred, and
the air sacs are not able to expand as much. As a result, not as
much oxygen can get to the body.
Interstitial lung disease (ILD) can occur without a known cause. This
is called idiopathic ILD. Idiopathic pulmonary fibrosis (IPF) is the
most common disease of this type.
There are also dozens of known causes of ILD, including:
• Autoimmune diseases (in which the immune system attacks the
body) such as lupus, rheumatoid arthritis, sarcoidosis, and
scleroderma
• Lung inflammation due to breathing in a foreign substance such as
certain types of dust, fungus, or mold (hypersensitivity
pneumonitis)
• Medicines (such as nitrofurantoin, sulfonamides, bleomycin,
amiodarone, methotrexate, gold, infliximab, and etanercept)
• Radiation treatment to the chest
• Working with or around asbestos, coal dust, cotton dust, and silica
dust (called occupational lung disease)
Cigarette smoking may increase the risk of developing some forms
of ILD and may cause the disease to be more severe.
Symptoms
Shortness of breath is a main symptom of interstitial lung disease.
You may breathe faster or need to take deep breaths:
• At first, shortness of breath may not be severe and is only noticed
with exercise, climbing stairs, and other activities.
• Over time, it can occur with less strenuous activity such as bathing
or dressing, and as the disease worsens, even with eating or
talking.
Most people with this condition also have a dry cough. A dry cough
means you do not cough up any mucus or sputum.
Over time, weight loss, fatigue, and muscle and joint pain are also
present.
People with more advanced ILD may have:
• Abnormal enlargement of the base of the fingernails (clubbing)
• Blue color of the lips, skin, or fingernails due to low blood oxygen
levels (cyanosis)
• Symptoms of the other diseases such as arthritis, associated with
ILD
Exams and Tests
The health care provider will perform a physical exam. Dry, crackling
breath sounds may be heard when listening to the chest with a
stethoscope.
The following tests may be done:
• Blood tests to check for autoimmune diseases
• Bronchoscopy with or without biopsy
• Chest x-ray
• CT scan of the chest
• Echocardiogram
• Open lung biopsy
• Measurement of the blood oxygen level at rest or when active
• Blood gases
• Pulmonary function tests
• Six minute walk test (checks how far you can walk in 6 minutes
and how many times you need to stop to catch your breath)
People who are heavily exposed to known causes of lung disease in
the workplace are usually routinely screened for lung disease. These
jobs include coal mining, sand blasting, and working on a ship.
Treatment
Treatment depends on the cause of the disease. Medicines that
suppress the immune system and reduce swelling in the lungs are
prescribed if an autoimmune disease is causing the problem. For
some people who have IPF, pirfenidone and nintedanib are two
medicines that may be used to slow the disease. If there is no
specific treatment for the condition, the aim is to make you more
comfortable and support lung function:
• If you smoke, ask your provider about how to stop smoking.
• People with low blood oxygen levels will receive oxygen therapy in
their home. A respiratory therapist will help you set up the
oxygen. Families need to learn proper oxygen storage and
safety.
Lung rehabilitation can provide support, and help you learn:
• Different breathing methods
• How to set up your home to save energy
• How to eat enough calories and nutrients
• How to stay active and strong
Some people with advanced ILD may need a lung transplant.
Support Groups
You can ease the stress of illness by joining a support group.
Sharing with others who have common experiences and problems
can help you not feel alone.
Outlook (Prognosis)
The chance of recovering or ILD getting worse depends on the
cause and how severe the disease was when it was first diagnosed.
Some people with ILD develop heart failure and high blood pressure
in the blood vessels of their lungs.
Idiopathic pulmonary fibrosis has a poor outlook.
When to Contact a Medical Professional
Call your provider if:
• Your breathing is getting harder, faster, or more shallow than
before
• You cannot get a deep breath, or need to lean forward when sitting
• You are having headaches more often
• You feel sleepy or confused
• You have a fever
• You are coughing up dark mucus
• Your fingertips or the skin around your fingernails is blue
Alternative Names
Diffuse parenchymal lung disease; Alveolitis; Idiopathic pulmonary
pneumonitis (IPP)
References
Raghu G. Interstitial lung disease. In: Goldman L, Schafer AI, eds.
Goldman's Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier
Saunders; 2016:chap 92.
Ryu JH, Selman M, Colby TV, King TE. Idiopathic interstitial
pneumonias. In: Broaddus VC, Mason RJ, Ernst JD, et al, eds.
Murray and Nadel's Textbook of Respiratory Medicine. 6th ed.
Philadelphia, PA: Elsevier Saunders; 2016:chap 63.
