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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.