Idiopathic Infiltrative Lung ... Health Article

Treatment

The first medication given, providing scarring is not too extensive, is usually a steroid drug such as prednisone. An occasional patient will improve dramatically if steroid therapy stops the inflammation. Most patients, however, improve to a limited extent. It may take 6–12 weeks for a patient to begin to respond. Patients must be watched closely for a gain in body weight, high blood pressure, and depression. Steroids can also result in diabetes, ulcer disease, and cataract. Patients treated with steroids are at risk of contracting serious infection. If steroids have not proved effective or have caused serious side effects, other anti-inflammatory drugs, such as cyclophosphamide (Cytoxan) or azathioprine (Imuran), can be tried. Cytoxan sometimes is combined with a steroid, but it carries its own risks, which include bladder inflammation and suppression of the bone marrow. Some patients will benefit from a bronchodilator drug that relaxes the airway and makes breathing easier.

Some patients with interstitial lung disease, especially children, will need oxygen therapy. Usually oxygen is given during sleep or exercise, but if the blood oxygen level is very low it may be given constantly. A program of conditioning, training in how to breathe efficiently, energy-saving tips, and a proper diet will help patients achieve the highest possible level of function given the state of their illness. All patients should be vaccinated each year against influenza. A last resort for those with very advanced disease who do not respond to medication is lung transplantation. This operation is being done more widely, and it is even possible to replace both lungs.

Prognosis

A scoring system based on lung function and x ray appearances has been designed to help monitor a patient's course. In general, idiopathic forms of interstitial lung disease cause a good deal of illness, and a significant number of deaths. A majority of patients get worse over time, although survival for many years is certainly possible. An estimated one in five affected children fail to survive. In different series, survival times average between four and ten years. Early diagnosis gives the best chance of a patient recovering or at least stabilizing. Once the lungs are badly scarred, nothing short of lung transplantation offers hope of restoring lung function. Patients with desquamative interstitial pneumonitis tend to respond well to steroid treatment, and live longer than those with other types of infiltrative lung disease.

Prevention

Since we do not understand what causes idiopathic interstitial lung diseases, there is no way to prevent them. What can be done is to prevent extensive scarring of the lungs by making the diagnosis shortly after the first symptoms develop, and trying steroids or other drugs in hope of suppressing lung inflammation. Every effort should be made to avoid exposure to dusts, gases, chemicals, and even pets. Keeping fit and learning how to breathe efficiently will help maintain lung function as long as possible.

BOOKS

Berkow, Robert, ed. Merck Manual of Medical Information: Home Edition. Whitehouse Station, NJ: Merck Research Laboratories, 1997.

ORGANIZATIONS

American Lung Association. 1740 Broadway, New York, NY 10019. (800) 586-4872. <http://www.lungusa.org>.

OTHER

"Pulmonary Medicine." Healthweb Page. 12 Jan. 1998 <http://healthweb.org/browse.cfm?subjectid=81>.

David A. Cramer, MD

KEY TERMS

Bronchoalveolar lavage—A way of obtaining a sample of fluid from the airways by inserting a flexible tube through the windpipe. Used to diagnose the type of lung disease.

Desquamation—Shedding of the cells lining the insides of the air sacs. A feature of desquamative interstitial pneumonitis.

Idiopathic—A disease whose cause is unknown.

Immune system—A set of body chemicals and specialized cells that attack an invading agent (such as a virus) by forming antibodies that can engulf and destroy it.

Infiltrative—A process whereby inflammatory or other types of disease spread throughout an organ such as the lungs.

Interstitial—Refers to the connective tissue that supports the "working parts" of an organ, in the case of the lungs the air sacs.

Pulmonary fibrosis—A scarring process that is the end result of many forms of long-lasting lung disease.