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$Unique_ID{BRK04113}
$Pretitle{}
$Title{Pneumonia, Interstitial}
$Subject{Pneumonia, Interstitial Chronic Fibrous Pneumonia Usual Interstitial
Pneumonia (UIP), also known as Idiopathic Interstitial Pneumonia or Fibrous
Interstitial Pneumonia Desquamative Interstitial Pneumonia (DIP) Giant-cell
Interstitial Pneumonia (GIP) Lymphoid Interstitial Pneumonia (LIP) Fibrosing
Alveolitis Extrinsic Allergic Alveolitis Idiopathic Pulmonary Fibrosis}
$Volume{}
$Log{}
Copyright (C) 1989 National Organization for Rare Disorders, Inc.
648:
Pneumonia, Interstitial
** IMPORTANT **
It is possible that the main title of the article (Interstitial
Pneumonia) is not the name you expected. Please check the SYNONYM listing to
find the alternate names and disorder subdivisions covered by this article.
Synonyms
Chronic Fibrous Pneumonia
Disorder Subdivisions:
Usual Interstitial Pneumonia (UIP), also known as Idiopathic Interstitial
Pneumonia or Fibrous Interstitial Pneumonia
Desquamative Interstitial Pneumonia (DIP)
Giant-cell Interstitial Pneumonia (GIP)
Lymphoid Interstitial Pneumonia (LIP)
Information on the following diseases can be found in the Related
Disorders section of this report:
Fibrosing Alveolitis
Extrinsic Allergic Alveolitis
Idiopathic Pulmonary Fibrosis
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.
Interstitial Pneumonia, involving the spaces and tissue (interstices) of
the lungs, is a type of primary pneumonia. It involves an abnormal increase
in the interstitial tissue and a decrease and hardening (induration) of other
lung tissue. Major symptoms may include shortness of breath on exertion,
cough (which may or may not be present) and loss of appetite.
Symptoms
Symptoms of Interstitial Pneumonia may vary from mild to severe according to
the extent of lung involvement, accumulation of tissue and cells not normally
found in the lungs (infiltrate), the rate of progress, and the presence of
complications (such as other lung infections). The patient often has no
fever (afebrile). Occasionally, however, the onset may be rapid, with
fever, suggesting an acute respiratory infection. Symptoms of lung disease
may be few, except for shortness of breath on exertion (exertional dyspnea).
Cough is not normally a prominent feature nor is overproduction of mucous;
these symptoms are more likely to be present when there is a secondary airway
(bronchial) infection. Blue coloring of the skin, fingernails and around the
lips (cyanosis) may occur, signaling a low level of oxygen in the blood.
Loss of appetite (anorexia), weight loss, fatigue, weakness, and vague chest
pains may also be present.
DISORDER SUBDIVISIONS
The following disorders are different types of interstitial pneumonias:
In Usual (or Idiopathic) Interstitial Pneumonia (or Fibrous) a pattern of
cellular accumulations (infiltrate), considerable scarring (fibrosis) and
destruction of the air cells (alveoli) accompanies an inflammatory reaction.
Usual Interstitial Pneumonia (UIP) probably represents a late stage
interstitial disease.
Desquamative Interstitial Pneumonia (DIP) is characterized by a marked
increase in alveolar cells with a small amount of scarring (minimal
interstitial fibrosis). It is generally viewed as a type of inflammation of
the alveoli (alveolitis) which can be caused by a variety of factors. It may
also represent an early stage of idiopathic (unknown) pulmonary fibrosis.
Discussion continues on whether these tissue changes represent different
diseases or "stages" of the same disease. Scientific evidence supports both
views.
Lymphoid Interstitial Pneumonia (LIP) maybe an autoimmune disease.
Autoimmune diseases occur when the body's natural defenses, such as
antibodies begin to attack healthy tissues. This type of interstitial
pneumonia also includes lymph system (lymphomatous) changes.
Some cases of Giant Cell Interstitial Pneumonia (GIP) are known to be
associated with exposure to hard metals particularly tungsten and cobalt.
Causes
The causes of the different interstitial pneumonias are not well understood.
Each type can be precisely diagnosed by the study of the lung tissue
(histology). Unlike many other types of pneumonia, the interstitial
pneumonias do not appear to be caused by bacteria.
Affected Population
Interstitial Pneumonia affects slightly more men than women. It has
generally been reported within an age range between 12 and 63 years.
Related Disorders
Symptoms of the following disorders can be similar to those of Interstitial
Pneumonia. Comparisons may be useful for a differential diagnosis:
Fibrosing Alveolitis is an inflammatory lung disorder characterized by
abnormal formation of fibrous tissue between the alveoli or ducts in the
lungs. Coughing and cyanosis may occur. (For more information on this
disorder, choose "Alveolitis, Fibrosing" as your search term in the Rare
Disease Database).
Extrinsic Allergic Alveolitis is a lung disorder resulting from repeated
inhalation of organic dust, usually in a work setting. (For more information
on this disorder, choose "Alveolitis, Extrinsic Allergic" as your search term
in the Rare Disease Database).
Idiopathic Pulmonary Fibrosis is an inflammatory disease of the lung
tissue characterized by cellular infiltration, an interstitial thickening,
and may involve the alveoli, blood vessels, airways (bronchi) and membranes
surrounding the lungs (pleura). The cause is unknown.
Therapies: Standard
Treatment of Interstitial Pneumonia is usually with corticosteroid drugs in
patients who have no evidence of extensive fibrosis. Oxygen may be
prescribed in cases of lack of oxygen levels in the blood (hypoxemia).
Antibiotics are required if secondary bacterial infection occurs. Other
treatment is symptomatic and supportive.
Therapies: Investigational
This disease entry is based upon medical information available through April
1989. 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 Interstitial Pneumonia, 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
INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and
Co., 1987. Pp. 661.
PULMONARY DISEASES AND DISORDERS, 2nd. Ed.: Alfred P. Fishman, M.D.;
McGraw-Hill, Inc., 1988. Pp. 739, 959, 1380, 756, 1684.
GIANT CELL INTERSTITIAL PNEUMONIA IN A HARD-METAL WORK. CYTOLOGIC,
HISTOLOGIC AND ANALYTICAL ELECTRON MICROSCOPIC INVESTIGATION. V.L. Roggli,
et. al.; Acta Cytol. (Mar-Apr, 1988, issue 32(2)). Pp. 240-6.
DESQUAMATIVE INTERSTITIAL PNEUMONIA. COMPUTED TOMOGRAPHIC FINDINGS
BEFORE AND AFTER TREATMENT WITH CORTICOSTEROIDS. S. Vedal, et. al.; Chest.
(Jan., 1988, issue 93(1)). Pp. 215-7.
LYMPHOCYTIC INTERSTITIAL PNEUMONIA. A.S. Teirstein, et. al.; Clin Chest
Med. (Sep., 1988, issue 9(3)). Pp. 467-71.