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Treatment of Wegener's Granulomatosis With Immunosuppressive Agents: Description of Renal Ultrastructure

Treatment of Wegener's Granulomatosis With Immunosuppressive Agents: Description of Renal... Abstract More than 100 cases of Wegener's granulomatosis have been described since the original reports of Klinger in 1931 and Wegener in 1936. Clinically, the disease is characterized by a severe progressive sinusitis; cough; and finally, in the majority of cases, by renal failure.1,2 Pathologically it has been characterized by necrotizing granulomatous lesions in the upper or lower respiratory tract, focal glomerulitis, and generalized focal necrotizing vasculitis.3-5 The prognosis in Wegener's granulomatosis has been extremely poor, despite the use of antibiotics, radiation therapy, and steroids.2,4,6 In the last seven years, however, there have been scattered reports of its successful treatment with alkylating or immunosuppressive agents. Since there is evidence that this process may be related to an immunologic or hypersensitivity state,1,5 the use of immunosuppression seems justified. Patient Summary A 29-year-old white man (467 044) was admitted to university hospital May 13, 1968, with a chief complaint References 1. Fahey JL, Leonard E, Churg J, et al: Wegener's granulomatosis. Amer J Med 17:168-179,1954.Crossref 2. Walton WE: Giant-cell granuloma of the respiratory tract (Wegener's granulomatosis). Brit Med J 2:265-270, 1958.Crossref 3. Godman GC, Churg J: Wegener's granulomatosis: Pathology and review of literature. Arch Path 58:533-553, 1954. 4. Sproul EE: Wegener's and lethal midline granulomatosis. JAMA 177:921-922,1961.Crossref 5. Tuhy JE, Maurice GL, Niles NR: Wegener's granulomatosis. Amer J Med 25:638-646,1958.Crossref 6. Merrill MD: Roentgen therapy in Wegener's granulomatosis. Amer J Roentgen 85:96-98,1961. 7. Györkey F, Min K-W, Sincovics JG, et al: Systemic lupus erythematosus and myxovirus. New Eng J Med 280:333,1969. 8. Bischoff ME: Noninfectious necrotizing granulomatosis: Pulmonary roentgen signs. Radiology 75:752-756,1960.Crossref 9. Felson B: Less familiar roentgen patterns of pulmonary granulomas. Amer J Roentgen 81:211-223,1959. 10. Norton WL, Suki W, Strunk S: Combined corticosteroid and azathioprine therapy in 2 patients with Wegener's granulomatosis. Arch Intern Med 121:554-560,1968.Crossref 11. Carrington CB, Liebow AA: Limited forms of angiitis and granulomatosis of Wegener's type. Amer J Med 41:497-527,1966.Crossref 12. Blatt IM, Holbrooke SS, Rubin P, et al: Fatal granulomatosis of the respiratory tract (lethal midline granuloma-Wegener's granulomatosis). Arch Otolaryng 70:707-748,1959.Crossref 13. Fred HL, Lynch EC, Greenberg SD, et al: A patient with Wegener's granulomatosis exhibiting unusual clinical and morphological features. Amer J Med 37:311-319,1964.Crossref 14. Beidleman B: Wegener's granulomatosis, prolonged therapy with large doses of steroids. JAMA 186:827-830, 1963.Crossref 15. Stern GM, Hoffbrand AV, Urich H: Peripheral nerves and skeletal muscles in Wegener's granulomatosis. Brain 88:151-164,1965.Crossref 16. Evans DW, Halley JB: Wegener's granulomatosis: Report of a patient surviving four and a half years. J Clin Path 16:215-219, 1963.Crossref 17. Hollander D, Manning RT: The use of alkylating agents in the treatment of Wegener's granulomatosis. Ann Intern Med 67:393-398, 1967.Crossref 18. Aungst CW, Lessmann EM: Wegener's granulomatosis treated with nitrogen mustard. New York J Med 62:3302-3310,1962. 19. Von Leden H, Schiff M: Antimetabolite therapy in lethal midline granuloma. Arch Otolaryng 80:460-468,1964.Crossref 20. Greenspan EM: Cyclophosphamideprednisone therapy of lethal midline granuloma. JAMA 193:74-76,1965.Crossref 21. Bouroncle BA, Smith EJ, Cuppage FE: Treatment of Wegener's granulomatosis with Imuran. Amer J Med 42:314-318, 1967.Crossref 22. McIlvanie SK: Wegener's granulomatosis: Successful treatment with chlorambucil. JAMA 197:90-92, 1966.Crossref 23. Kaplan SR, Hayslett JP, Calabresi P: Treatment of advanced Wegener's granulomatosis with azathioprine and duazomycin A. New Eng J Med 278:239-244,1968.Crossref 24. Choy DSJ, Gould WJ, Gearhart RP, et al: Remission in Wegener's granulomatosis treated with steroids and Imuran. New York J Med 69:1205-1209,1969. 25. Castleman B (ed): Wegener's granulomatosis case records of the Massachusetts General Hospital. New Eng J Med 280:828-834,1969.Crossref 26. Hood RM, O'Connel HV, McCord, DM: Pathergic granulomatosis (Wegener's granuloma) of the lung. Amer Rev Tuberc 78:21-37,1958. 27. Goodman, LS, Gilman A: The Pharmacologic Basis of Therapeutics . Toronto, MacMillan Co, 1965. 28. Schwartz RS: Therapeutic strategy in clinical immunology. New Eng J Med 280:367-374,1969.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Internal Medicine American Medical Association

Treatment of Wegener's Granulomatosis With Immunosuppressive Agents: Description of Renal Ultrastructure

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Publisher
American Medical Association
Copyright
Copyright © 1970 American Medical Association. All Rights Reserved.
ISSN
0003-9926
eISSN
1538-3679
DOI
10.1001/archinte.1970.00310080104017
Publisher site
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Abstract

Abstract More than 100 cases of Wegener's granulomatosis have been described since the original reports of Klinger in 1931 and Wegener in 1936. Clinically, the disease is characterized by a severe progressive sinusitis; cough; and finally, in the majority of cases, by renal failure.1,2 Pathologically it has been characterized by necrotizing granulomatous lesions in the upper or lower respiratory tract, focal glomerulitis, and generalized focal necrotizing vasculitis.3-5 The prognosis in Wegener's granulomatosis has been extremely poor, despite the use of antibiotics, radiation therapy, and steroids.2,4,6 In the last seven years, however, there have been scattered reports of its successful treatment with alkylating or immunosuppressive agents. Since there is evidence that this process may be related to an immunologic or hypersensitivity state,1,5 the use of immunosuppression seems justified. Patient Summary A 29-year-old white man (467 044) was admitted to university hospital May 13, 1968, with a chief complaint References 1. Fahey JL, Leonard E, Churg J, et al: Wegener's granulomatosis. Amer J Med 17:168-179,1954.Crossref 2. Walton WE: Giant-cell granuloma of the respiratory tract (Wegener's granulomatosis). Brit Med J 2:265-270, 1958.Crossref 3. Godman GC, Churg J: Wegener's granulomatosis: Pathology and review of literature. Arch Path 58:533-553, 1954. 4. Sproul EE: Wegener's and lethal midline granulomatosis. JAMA 177:921-922,1961.Crossref 5. Tuhy JE, Maurice GL, Niles NR: Wegener's granulomatosis. Amer J Med 25:638-646,1958.Crossref 6. Merrill MD: Roentgen therapy in Wegener's granulomatosis. Amer J Roentgen 85:96-98,1961. 7. Györkey F, Min K-W, Sincovics JG, et al: Systemic lupus erythematosus and myxovirus. New Eng J Med 280:333,1969. 8. Bischoff ME: Noninfectious necrotizing granulomatosis: Pulmonary roentgen signs. Radiology 75:752-756,1960.Crossref 9. Felson B: Less familiar roentgen patterns of pulmonary granulomas. Amer J Roentgen 81:211-223,1959. 10. Norton WL, Suki W, Strunk S: Combined corticosteroid and azathioprine therapy in 2 patients with Wegener's granulomatosis. Arch Intern Med 121:554-560,1968.Crossref 11. Carrington CB, Liebow AA: Limited forms of angiitis and granulomatosis of Wegener's type. Amer J Med 41:497-527,1966.Crossref 12. Blatt IM, Holbrooke SS, Rubin P, et al: Fatal granulomatosis of the respiratory tract (lethal midline granuloma-Wegener's granulomatosis). Arch Otolaryng 70:707-748,1959.Crossref 13. Fred HL, Lynch EC, Greenberg SD, et al: A patient with Wegener's granulomatosis exhibiting unusual clinical and morphological features. Amer J Med 37:311-319,1964.Crossref 14. Beidleman B: Wegener's granulomatosis, prolonged therapy with large doses of steroids. JAMA 186:827-830, 1963.Crossref 15. Stern GM, Hoffbrand AV, Urich H: Peripheral nerves and skeletal muscles in Wegener's granulomatosis. Brain 88:151-164,1965.Crossref 16. Evans DW, Halley JB: Wegener's granulomatosis: Report of a patient surviving four and a half years. J Clin Path 16:215-219, 1963.Crossref 17. Hollander D, Manning RT: The use of alkylating agents in the treatment of Wegener's granulomatosis. Ann Intern Med 67:393-398, 1967.Crossref 18. Aungst CW, Lessmann EM: Wegener's granulomatosis treated with nitrogen mustard. New York J Med 62:3302-3310,1962. 19. Von Leden H, Schiff M: Antimetabolite therapy in lethal midline granuloma. Arch Otolaryng 80:460-468,1964.Crossref 20. Greenspan EM: Cyclophosphamideprednisone therapy of lethal midline granuloma. JAMA 193:74-76,1965.Crossref 21. Bouroncle BA, Smith EJ, Cuppage FE: Treatment of Wegener's granulomatosis with Imuran. Amer J Med 42:314-318, 1967.Crossref 22. McIlvanie SK: Wegener's granulomatosis: Successful treatment with chlorambucil. JAMA 197:90-92, 1966.Crossref 23. Kaplan SR, Hayslett JP, Calabresi P: Treatment of advanced Wegener's granulomatosis with azathioprine and duazomycin A. New Eng J Med 278:239-244,1968.Crossref 24. Choy DSJ, Gould WJ, Gearhart RP, et al: Remission in Wegener's granulomatosis treated with steroids and Imuran. New York J Med 69:1205-1209,1969. 25. Castleman B (ed): Wegener's granulomatosis case records of the Massachusetts General Hospital. New Eng J Med 280:828-834,1969.Crossref 26. Hood RM, O'Connel HV, McCord, DM: Pathergic granulomatosis (Wegener's granuloma) of the lung. Amer Rev Tuberc 78:21-37,1958. 27. Goodman, LS, Gilman A: The Pharmacologic Basis of Therapeutics . Toronto, MacMillan Co, 1965. 28. Schwartz RS: Therapeutic strategy in clinical immunology. New Eng J Med 280:367-374,1969.Crossref

Journal

Archives of Internal MedicineAmerican Medical Association

Published: Aug 1, 1970

References