$Unique_ID{BRK03447} $Pretitle{} $Title{Alveolitis, Fibrosing} $Subject{Alveolitis, Fibrosing Interstitial Diffuse Pulmonary Fibrosis Pulmonary Fibrosis, Idiopathic Diffuse Fibrosing Alveolitis Alveolocapillary block Cryptogenic Fibrosing Alveolitis Hamman-Rich Syndrome Interstitial Pneumonia Extrinsic Allergic Alveolitis} $Volume{} $Log{} Copyright (C) 1987, 1989 National Organization for Rare Disorders, Inc. 432: Alveolitis, Fibrosing ** IMPORTANT It is possible the main title of the article (Fibrosing Alveolitis) is not the name you expected. Please check the SYNONYMS listing on the next page to find alternate names, disorder subdivisions, and related disorders covered by this article. Synonyms Interstitial Diffuse Pulmonary Fibrosis Pulmonary Fibrosis, Idiopathic Diffuse Fibrosing Alveolitis Alveolocapillary block Cryptogenic Fibrosing Alveolitis Hamman-Rich Syndrome Information on the following diseases can be found in the Related Disorders section of this report: Interstitial Pneumonia Extrinsic Allergic Alveolitis General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources section of this report. Fibrosing Alveolitis is an inflammatory lung disorder characterized by abnormal formation of fibrous tissue between tiny air sacs (alveoli) or ducts in the lungs. Coughing and rapid, shallow breathing occur with moderate exercise. The skin may appear slightly bluish (cyanotic) due to lack of circulating oxygen. Complications such as infection, emphysema or heart problems may develop. Symptoms Fibrosing Alveolitis is marked by progressive breathing difficulty and coughing which may not ease the bronchial irritation. Loss of appetite, weight loss, fatigue, weakness, fever, and vague chest pains may be common. Ends of the fingers or toes may become broadened and shiny (clubbed). When oxygen circulation in the blood is poor, the skin may appear bluish (cyanotic). Infections can easily occur due to a weakened immune system. Complications including emphysema, pulmonary infections, or heart disease may develop in untreated patients. Severity of symptoms may vary widely. Causes The exact cause of Fibrosing Alveolitis is not known. Researchers believe that Scleroderma (changes in collagen tissue in the lungs), a blood factor associated with rheumatoid arthritis, or an autoimmune factor may be causes. Autoimmune disorders are caused when the body's natural defenses (antibodies) suddenly begin to attack healthy tissue. Affected Population Fibrosing Alveolitis affects males and females in equal numbers, usually during middle age. Related Disorders Symptoms of the following lung disorders can be similar to those of Fibrosing Alveolitis. Comparisons may be useful for a differential diagnosis: Interstitial Pneumonia is characterized by breathing difficulty and is thought to be a variation of Fibrosing Alveolitis. It can begin gradually. The abnormal lung tissue growths tend to be more uniform than those of Fibrosing Alveolitis. This disorder usually has a favorable response to treatment with systemic corticosteroid drugs. Extrinsic Allergic Alveolitis is a lung disorder similar to Fibrosing Alveolitis. It is caused by repeated or constant exposure to organic substances which usually produce short-term, mild episodes of breathlessness associated with lung irritation or allergic reactions. However, repeated attacks tend to be progressively more severe, and may be characterized by fever, breathing difficulty, crackling sounds during breathing (rales), bluish appearance of the skin (cyanosis), and blood in the sputum. (For more information on this disorder, choose "Alveolitis" as your search term in the Rare Disease Database.) Therapies: Standard Treatment of Fibrosing Alveolitis with systemic corticosteroid drugs may prevent lung changes before they become widespread or permanent. High doses may be recommended at first, followed by a lower maintenance dosage. Some cases which prove resistant to steroid therapy may improve with the purine antagonist drug, azathioprine. Imaging techniques may be useful to monitor progressive lung changes. Oxygen administered in high concentrations may be helpful if oxygen in the blood is diminished. Antibiotics may be required if bacterial infections develop. Digitalis or diuretic drugs may be recommended if heart problems arise. Other treatment is symptomatic and supportive. Therapies: Investigational Lung transplants are under investigation as a possible treatment for Fibrosing Alveolitis. Effectiveness and side effects of this procedure have not been fully documented and more extensive research is being pursued. This disease entry is based upon medical information available through February 1988. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Fibrosing Alveolitis, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 American Lung Association 1740 Broadway New York, NY 10019 (212) 315-8700 NIH/National Heart, Lung and Blood Institute (NHLBI) 9000 Rockville Pike Bethesda, MD 20892 (301) 496-4236 References EFFECT OF INTERMITTENT HIGH DOSE PARENTERAL CORTICOSTEROIDS ON THE ALVEOLITIS OF IDIOPATHIC PULMONARY FIBROSIS: B.A. Keogh, et al.; Am Rev Respir Dis (January 1983, issue 127(1)). Pp. 18-22. BRONCHOALVEOLAR LAVAGE FLUID NEUTROPHILS INCREASE AFTER CORTICOSTEROID THERAPY IN SMOKERS WITH IDIOPATHIC PULMONARY FIBROSIS: K.L. Christopher, et al.; Am Rev Respir Dis (January 1986, issue 133(1)). Pp. 104-109. CONCENTRATION, BIOSYNTHESIS AND DEGRADATION OF COLLAGEN IN IDIOPATHIC PULMONARY FIBROSIS: M. Selman, et al.; Thorax (May 1986, issue 41(5)). Pp. 355-359.