Interstitial Lung Disease (ILD)

Interstitial lung disease (ILD) is a general term used to describe a large collection of lung diseases.  It is a very complex area of lung medicine.  The specific lung diseases are often referred to by abbreviations which sound like alphabet soup.  For example, IPF, UIP, NSIP, LIP, DIP, RBILD, HP, EG are just a sampling. 

The major symptom of ILD is shortness of breath.  Cough is also common.  Often patients have had symptoms for months to years before an accurate diagnosis is made.  It is also common to have been treated repeatedly for pneumonia or asthma before a referral to a lung specialist is made. 

Some of these diseases are associated with cigarette smoking.  Your doctor will take a careful history of your work experience and hobbies as certain things in your environment can trigger some of these diseases.  For example, asbestos exposure is known to cause a form of pulmonary fibrosis. 

The evaluation of ILD is complex and involves many tests.  For example, most patients will need a full battery of breathing tests (pulmonary function tests-PFTs), special chest CT scans, blood work and a walking test.  Two tests in particular are important in many patients with ILD—Bronchoscopy and VATS (video assisted thoracic surgery, a type of lung biopsy).  Bronchoscopy is a painless procedure that is used to look for infection or inflammation in the lung.  Very small biopsies can be taken.  Surgical lung biopsy is the gold standard for the diagnosis of most ILDs. 

If you are a smoker it is very important to stop smoking.  Also, we encourage annual influenza vaccinations and pneumonia vaccinations in patients with ILD.

It is very important for your lung doctor to try and determine which specific type of lung biopsy you have.  Treatment depends on the specific disease and often involves very strong medicines with side effects.  In some patients, lung transplantation is an important option.  Recently approved medications now allow us to offer patients with Idiopathic Pulmonary Fibrosis effective therapy for the first time.   

The lung doctors at Arizona Pulmonary Specialists are experts in the diagnosis and treatment of interstitial lung disease.  Our team of doctors, nurses and respiratory therapists will work with you to ensure that you understand your diagnosis, medications and other treatments. 

At Arizona Pulmonary Specialists we are actively involved in many research studies.  We often have studies of new medications for ILD.  Ask your doctor if you are interested.


UIP:  usual interstitial pneumonia 
IPF:  idiopathic pulmonary fibrosis
NSIP:  non-specific interstitial pneumonitis
LIP:  lymphocytic pneumonitis
DIP:  desquamative interstitial pneumonitis
RBILD: respiratory bronchiolitis interstitial lung disease
HP:  hypersensitivity pneumonitis
EG:  eosinophilic granuloma (also referred to as histiocytosis X and Langerhans cell histiocytosis)