home *** CD-ROM | disk | FTP | other *** search
- $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.
-
-