Dr. M.J. Bazos, MD Patient Handout

About Your Diagnosis
The term “diffuse interstitial pulmonary disease” is a collective name for a group of many different lung diseases that have varying causes and manifestations. These diseases are linked together because they all cause inflammation of the area surrounding the air sacs of the lungs. This inflammation can result from inhalation of environmental or job-related irritants, medications, radiation, inflammatory diseases outside the lung, or for unknown reasons. The inflammation often leads to lung scarring (fibrosis) that significantly impairs lung function. These conditions are not contagious. There are no preventive vaccines, and they are generally difficult to treat because cures are not available in most circumstances. A typical interstitial lung disease is idiopathic pulmonary fibrosis. The term “idiopathic” means that the cause of the lung scarring is unknown. This is a chronic disease of middle-aged men and women that is characterized by a steady decline in exercise tolerance as the lung scarring progresses. Fifty percent of patients will die within 3–5 years of the onset of chest symptoms. Some patients will decline rapidly, whereas others will stabilize over time. Spontaneous remissions are very rare. Diagnosis begins with a history and physical examination conducted by your health care provider. Questions will be asked regarding your job history, smoking history, whether you have had diseases outside the chest, the nature of any prior medical treatments, and whether there is a family history of interstitial lung disease. With lung scarring, the physical examination may reveal crackling breath sounds, which resemble the sound made by a piece of Velcro being released. The fingertips may take on a clubbed-like appearance. The chest x-ray is usually abnormal and can reveal a variety of changes, including a honeycomb-like appearance. A chest computed tomography (CT) scan is often obtained to further define the patterns of lung inflammation. If the diagnosis remains unclear, biopsy specimens can be obtained by bronchoscopy, a procedure by which your doctor uses a lighted tube to look down into your lungs. It may be necessary for a chest surgeon to perform the lung biopsy while the patient is receiving general anesthesia. Breathing tests are performed to assess how the interstitial lung disease is affecting function.

Living With Your Diagnosis
Because interstitial lung disease includes a group of many different diseases, the symptoms may vary. However, nearly all produce shortness of breath and
dry cough that usually start gradually and slowly worsen over time. In those cases where the lung inflammation is part of a disease affecting other organs, symptoms outside the lung may predominate. Abrupt chest pain may signal lung collapse. Fever and changes in cough raise the possibility of bacterial or viral pneumonia. Coughing up blood may occur with some interstitial lung diseases or may be caused by a cancer. Heart failure may occur as the illness worsens.

Treatment
Treatment options vary depending on the specific interstitial lung disease. Smoking cessation is recommended in all cases and may be all that is necessary with some diseases. Inhalers may be prescribed to open the bronchial tubes if there is also smoking-related lung disease. If the interstitial lung disease is felt to be caused by environment exposures, avoidance measures are indicated (such as working with protective breathing masks). Frequently, a decision must be made about the use of potent anti-inflammatory drugs. The most commonly prescribed drug in this regard is the steroid, prednisone. Side effects include:
• Rapid mood swings.
• Weight gain resulting from water retention and a strong appetite.
• Facial puffiness.
• Easy bruising, especially over hands and forearms.
• High blood pressure.
• Poor blood sugar control.
• Cataracts.
• Bone loss.
• Increased susceptibility to infections.
Because the interstitial lung diseases are potentially life threatening, your doctor may feel that the benefits of using drugs like prednisone outweigh the risks. Months to years of therapy may be necessary, but generally the prednisone dose is tapered to the lowest possible dose. The prednisone may be combined with other anti-inflammatory agents. Response to therapy is judged by changes in symptoms, chest x-ray findings, and breathing tests. Unfortunately, treatment of lung fibrosis is often unrewarding. Treatment options are limited, and there are no drugs that reliably reverse lung scarring. Supplemental oxygen may be used to decrease shortness of breath and improve stamina. Lung transplantation is considered in certain patients.

The DOs
• Obtain an influenza vaccination each fall.
• Obtain/update the pneumococcal vaccination.
• Maintain good cardiovascular fitness by participating in an exercise program.
• Maintain close contact with your health care provider.

The DON’Ts
• Avoid all further exposures to any inhaled material felt to be responsible for your interstitial lung disease.
• Avoid individuals with acute respiratory infections.
• Stop smoking.

When to Call Your Doctor
Call your doctor if any of the following occur:
• If you suspect that you have a lung infection as suggested by an abrupt worsening of cough, yellow or green phlegm production, increased shortness of breath, fevers, or chills.
• Blood in your sputum.
• Dusky-colored skin, fingertips, or lips.
• Chest pain.
• New ankle swelling.
• Any problems with medications.

Websites:
American Lung Association
www.lungusa.org