$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.