$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.