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- $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.
-
-