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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.