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- $Unique_ID{BRK03785}
- $Pretitle{}
- $Title{Granulomatosis, Lymphomatoid}
- $Subject{Granulomatosis Lymphomatoid Pulmonary Angiitis Pulmonary Wegener's
- Granulomatosis Benign Lymph Angiitis and Granulomatosis Malignant Lymph
- Angiitis and Granulomatosis Wegener's Granulomatosis Churg-Strauss Syndrome
- Sarcoidosis Lymphoma}
- $Volume{}
- $Log{}
-
- Copyright (C) 1990 National Organization for Rare Disorders, Inc.
-
- 717:
- Granulomatosis, Lymphomatoid
-
- ** IMPORTANT **
- It is possible that the main title of the article (Lymphomatoid
- Granulomatosis) is not the name you expected. Please check the SYNONYM
- listing to find the alternate names and disorder subdivisions covered by this
- article.
-
- Synonyms
-
- Pulmonary Angiitis
- Pulmonary Wegener's Granulomatosis
- Benign Lymph Angiitis and Granulomatosis
- Malignant Lymph Angiitis and Granulomatosis
-
- Information on the following diseases can be found in the Related
- Disorders section of this report:
-
- Wegener's Granulomatosis
- Churg-Strauss Syndrome
- Sarcoidosis
- Lymphoma
-
- 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.
-
- Lymphomatoid Granulomatosis is a rare, progressive, vascular disease
- characterized by infiltration and destruction of the veins and arteries by
- lesions. These lesions can affect various parts of the body, especially the
- lungs. It can be a benign or malignant condition.
-
- Symptoms
-
- Lymphomatoid Granulomatosis is a serious disease characterized by the
- infiltration and destruction of the veins and arteries by nodular lesions
- that can affect the lungs, skin, kidneys or central nervous system. There
- may be a cough with or without blood, fever, weight loss, diarrhea, joint
- (arthralgias) and muscle (myalgias) pain, shortness of breath (dyspnea),
- chest pain and a generalized feeling of discomfort (malaise). If the skin is
- involved, flat and red lesions (macules), nodules and sometimes ulcerations
- can appear.
-
- Lymphomatoid Granulomatosis is a progressive disease that can lead to
- breathing difficulties and eventually failure of the respiratory system. In
- some severe cases, the nodular lesions can change and take on the malignant
- characteristics of lymphoma. A lung biopsy may be helpful in determining a
- proper diagnosis.
-
- Causes
-
- The exact cause of Lymphomatoid Granulomatosis is unknown. Some cases of
- this disease may be caused by an allergic reaction to an unknown antigen.
- Others appear to be an autoimmune disorder. Autoimmune disorders occur when
- the body's natural defenses (antibodies, lymphocytes, etc.) against invading
- organisms suddenly begin to attack healthy tissue.
-
- Affected Population
-
- Lymphomatoid Granulomatosis can occur at any age. It is seen more often
- after the age of forty and is slightly more common in males than females.
-
- Related Disorders
-
- Symptoms of the following disorders can be similar to those of Lymphomatoid
- Granulomatosis. Comparisons may be useful for a differential diagnosis:
-
- Wegener's Granulomatosis is an uncommon collagen vascular disorder that
- begins as a localized inflammation of the upper and lower mucous membranes in
- the respiratory tract. It usually progresses into generalized inflammation of
- the blood vessels (vasculitis) and kidney (glomerulonephritis). (For more
- information on this disorder, choose "Wegener" as your search term in the
- Rare Disease Database).
-
- Churg-Strauss Syndrome is a lung disorder often occuring as a
- complication of other disorders. Allergic blood vessel inflammation (angiitis
- or vasculitis) is accompanied by many inflammatory nodular lesions
- (granulomatosis) which may be small or granular, and are made up of compactly
- grouped cells. (For more information on this disorder, choose "Churg" as
- your search term in the Rare Disease Database.
-
- Sarcoidosis is a disorder which affects many body systems. It is
- characterized by small round lesions (tubercles) of granulation tissue.
- Symptoms may vary depending on the severity of the disease and how much of
- the body is affected. (For more information on this disorder, choose
- "Sarcoidosis" as your search term in the Rare Disease Database.)
-
- Lymphoma is a malignant growth in the lymph and reticuloendothelial
- systems of the body. It occurs most often in the lymph nodes, spleen and
- other areas involved in the body's immune system (lymphoreticular).
-
- Therapies: Standard
-
- Treatment of Lymphomatoid Granulomatosis consists of corticosteroid drugs
- and/or the drug cyclophosphamide. Other treatment is symptomatic and
- supportive.
-
- Therapies: Investigational
-
- For those cases where Lymphomatoid Granulomatosis has become malignant, the
- orphan drug prednimustine is being tested as a treatment for Lymphoma. (For
- more information on the Orphan Drug Prednimustine, physicians can contact:
-
- Smith-Kline & French Laboratories
- 1500 Spring Garden Street
- Philadelphia, PA 19101
-
- This disease entry is based upon medical information available through
- July 1990. 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 Lymphatoid Granulomatosis, 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
-
- American Cancer Society
- 1599 Clifton Rd., NE
- Atlanta, GA 30329
- (404) 320-3333
-
- NIH/National Heart, Lung and Blood Institute (NHLBI)
- 9000 Rockville Pike
- Bethesda, MD 20892
- (301) 496-4236
-
- NIH/National Cancer Institute
- 9000 Rockville Pike, Bldg. 31, Rm. 1A2A
- Bethesda, MD 20892
- 1-800-4-CANCER
-
- The National Cancer Institute has developed PDQ (Physicians Data Query),
- a computerized database designed to give the public, cancer patients and
- families, and health professionals quick and easy access to many types of
- information vital to patients with lymphoma and many other types of cancer.
- To gain access to this service, call:
- Cancer Information Service (CIS)
- 1-800-4-CANCER
- In Washington and suburbs in Maryland and Virginia, 636-5700
- In Alaska, 1-800-638-6070
- In Oahu, Hawaii, (808) 524-1234 (Neighbor islands call collect)
-
- References
-
- INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and
- Co., 1987. Pp. 665.
-
- PULMONARY DISEASES AND DISORDERS, Volume 2, 2nd. Ed.: Alfred P. Fishman
- M.D., ed.-in-chief; McGraw-Hill Book Co., 1980. Pp. 1127.
-
- BENIGN LYMPHOCYTIC ANGIITIS AND GRANULOMATOSIS. H. Tukianen, et al.;
- THORAX, (August 1988, issue 43 (8)). Pp.649-650.
-
- LYMPHOMATOID GRANULOMATOSIS: A REVIEW OF 12 CASES. J. Prenovault et al.;
- CAN ASSOC RADIOL J (December 1988, issue 39 (4)). Pp. 263-266.
-
- NECROTIZING VASCULITIS WITH GRANULOMATOSIS. I. Yevich; INT J DERMATOL,
- (October 1988, issue 27 (8)). Pp. 540-546.
-
-