Wegener's granulomatosis: Studies in eighteen patients and a review of the literature

Medicine (United States)

Volumn 52, Issue 6, 1973, Pages 535-561

  Fauci, Anthony S ^a   Wolff, Sheldon M ^a  

^a NATIONAL INSTITUTE OF ALLERGY AND INFECTIOUS DISEASES   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CYCLOPHOSPHAMIDE; IMMUNOSUPPRESSIVE AGENT;

CARDIOLOGY; DIAGNOSIS; EAR; ETIOLOGY; EYE; GLOMERULONEPHRITIS; HEART; HISTOLOGY; JOINT; KIDNEY; LUNG; MAJOR CLINICAL STUDY; NERVOUS SYSTEM; NOSE CAVITY; PARANASAL SINUS; RESPIRATORY SYSTEM; REVIEW; SKIN; THERAPY; VASCULITIS; WEGENER GRANULOMATOSIS;

ADULT; AGED; AZATHIOPRINE; BIOPSY; CORNEAL ULCER; CYCLOPHOSPHAMIDE; DIAGNOSIS, DIFFERENTIAL; EAR DISEASES; EYE MANIFESTATIONS; FEMALE; HUMAN; KIDNEY DISEASES; LUNG DISEASES; MALE; MIDDLE AGE; NASAL SEPTUM; NEUROLOGIC MANIFESTATIONS; PARANASAL SINUSES; SINUSITIS; SKIN MANIFESTATIONS; VASCULAR DISEASES; WEGENER'S GRANULOMATOSIS;

EID: 0015764397     PISSN: 00257974     EISSN: 15365964     Source Type: Journal    
DOI: 10.1097/00005792-197311000-00002     Document Type: Article

References (115)

1. Alarcön-Segovia, D. and Brown, A. L., Jr.: Classification and etiologio aspects of ne-crotizing angiitides. An analytic approach to a confused subject with a critical review of the evidence for hypersensitivity in poly-arteritis nodosa. Mayo Clin. Proa, 39: 305, 1964.
2. Aldo, M. A., Benson, M. D., Comerford, F. R. and Cohen, A. S.: Treatment of Wegener's granulomatosis with immunosuppressive agents. Arch. Intern. Med., 126: 298, 1970.
3. Allen, A. R. and Moen, C. W.: Wegener's granulomatosis. J. Thorac. Cardiovasc. Surg., 49: 388, 1965.
4. Allen, D. M., Diamond, L. K. and Howell, D. A.: Anaphylactoid purpura in children (Schönlein-Henoch syndrome): Review with a follow-up of the renal complications. Am. J. Dis. Child. 99: 833, 1960.
5. Aptekar, R. G., Atkinson, J. P., Decker, J. L., Wolff, S. M. and Chu, E. W.: Bladder tox-icity with chronic oral cyclophosphamide therapy in non-malignant disease. Arthritis Rheum., in press.
6. Athens, J. W., Haab, P. O., Raab, S. O., Mauer, A. M., Ashenbracker, H., Cartwright, G. E. and Wintrobe, M. M.: Leukokinetic studies. IV. The total blood, circulating and marginal granulocyte pools and the granulo-cyte turnover rate in normal subjects. J. Clin. Invest., 40: 989, 1961.
7. Atkins, J. B. and Eisman, S. H.: Wegener's granulomatosis. Ann. Otol., Rhinol., Laryngol., 68: 524, 1959.
8. Aungst, C. W. and Lessman, E. M.: Wegener's granulomatosis treated with nitrogen mustard. N.Y. State J. Med., 62: 3302, 1962.
9. Barr, J., Danielsson, D. and Magnusson, P.: Wegener's granulomatos, 3 fall-klinik och. immunofluorescens. Nord. Med., 86: 1291, 1971.
10. Beidelman, B.: Wegener's granulomatosis. Prolonged therapy with large dose of steroids. J.A.M.A., 186: 827, 1963.
11. Berman, D. A., Rydell, R. E. and Eiehenholtz, A.: Wegener's granulomatosis: clinicopatho-logic study of four cases. Ann. Intern. Med., 59: 521, 1963.
12. Blatt, I. M., Holbrooke, S. S., Rubin, P., Furstenberg, A. C., Maxwell, J. H. and Schull, W. J.: Fatal granulomatosis of the respiratory tract (lethal midline granuloma-Wegener's granulomatosis. A.M.A. Arch. Oto-laryngol., 70: 707, 1959.
13. Blatt, I. M. and Lawrence, M.: Otologic manifestations of fatal granulomatosis of respiratory tract. Arch. Otolaryngol., 73: 639, 1961.
14. Blodi, F. C. and Gass, J. D. M.: Inflammatory pseudotumor of the orbit. Brit. J. Ophthal., 52:79, 1968.
15. Bouroncle, B. A., Smith, E. J. and Cuppage, F. E.: Treatment of Wegener's granulomatosis with Imuran. Am. J. Med., 42: 314, 1967.
16. Brady, H. R., Israel, M. R. and Lewin, W. H.: Wegener's granulomatosis and corneo-scleral ulcer. J.A.M.A., 193: 248, 1965.
17. Breckenridge, R. L., Wagers, A. J. and Baltzell, W. H.: Lethal granuloma of the mid-line facial tissue: Granuloma gangraenscens. Ann. Otol., 63: 278, 1959.
18. Brooke, R. L: Wegeners' granulomatosis involving the gingivae. Brit. Dent. J., 127: 34, 1969.
19. Brubaker, R., Font, R. L. and Shepherd, E. M.: Granulomatous sclerouveitis. Arch. Ophthalmol., 86: 517, 1971.
20. Capizzi, R. L. and Bertino, J. R.: Methotrexate therapy of Wegener's granulomatosis. Ann. Intern. Med., 74: 74, 1971.
21. Carrington, C. B. and Liebow, A. A.: Limited forms of angiitis and granulomatosis of Wegener's type. Am. J. Med., 41: 497, 1966.
22. Cassan, S. M., Coles, D. T. and Harrison, E. G.: The concept of limited forms of Wegener's granulomatosis. Am. J. Med., 49: 366, 1970.
23. Cassan, S. M., Divertie, M. B., Hollenhorst, R. W. and Harrison, E. G.: Pseudotumor of the orbit in limited Wegener's granulomatosis. Ann. Intern. Med., 72: 687, 1970.
24. Cawson, R. A.: Gingival changes in Wegener's granulomatosis. Brit, Dent. J., 118: 30, 1965.
25. Choy, D. S., Gould, W. J., Gearhart, R. P. and Sauer, J.: Remission in Wegener's granulomatosis treated with steroids and azathio-prine. N.Y. State J. Med., 69: 1205, 1969.
26. Churg, J.: Allergic granulomatosis and granlomatous-vascular syndromes. Ann. Allergy 21: 619, 1963.
27. Churg, J. and Grishman, E.: Ultrastructure of immune deposits in renal glomeruli. Ann. Intern. Med., 76: 479, 1972.
28. Clarke, P. B.: Wegener's granulomatosis. Brit. J. Oral Surg., 1: 205, 1964.
29. Clinicopathological Conference. N. Engl. J. Med., 280: 828, 1969.
30. Crummy, C. S., Perlin, E. and Moquin, R. B.: Microangiopathic hemolytic anemia in Weg-ener's granulomatosis. Am. J. Med., 51: 544, 1971.
31. Dale, D. C., Fauci, A. S. and Wolff, S. M.: The effect of cyclophosphamide on leukocyte kinetics and susceptibility to infection in patients with Wegener's granulomatosis. Arthritis Rheum., in press.
32. DeOreo, G. A.: Wegener's granulomatosis. A.M.A. Arch. Dermatol., 81: 169, 1960.
33. Drachman, D. A.: Neurological complications of Wegener's granulomatosis. Arch. Neurol., 8: 145, 1963.
34. Eichel, B. S., Harrison, E. G., Jr., Devine, K. D., Scanlon, P. W. and Brown, H. A.: Primary lymphoma of the nose including relationship to lethal midline granuloma. Am. J. Surg., 112: 597, 1966.
35. Eichel, B. S. and Mabery, T. E.: The enigma of the lethal midline granuloma. Laryngoscope, 78: 1367, 1968.
36. Elsner, B. and Harper, F. B.: Disseminated Wegener's granulomatosis with breast involvement. Arch. Path., 87: 544, 1969.
37. Evans, D. W. and Halley, J. B. W.: Wegener's granulomatosis: A report of a patient surviving four and a half years. J. Clin. Path., 16: 215, 1963.
38. Fahey, J., Leonard, E., Churg, J. and Godman, G.: Wegener's granulomatosis. Am. J. Med., 17: 168, 1954.
39. Fairley, K. F., Barrie, J. U. and Johnson, W.: Sterility and testicular atrophy related to cyclophosphamide therapy. Lancet, 1: 568, 1972.
40. Fauci, A. S. and. Wolff, S. M.:. Treatment of Wegener's granulomatosis with cyclophosphamide. J. Clin. Invest., 50: 28a, 1971.
41. Fauci, A. S., Wolff, S. M. and Johnson, J. S.: Effect of cyclophosphamide upon the immune response in Wegener's granulomatosis. N. Engl. J. Med., 285: 1493, 1971.
42. Fechner, R. E. and Lamppin, D. W.: Midline malignant reticulosis. A clinicopathologic entity. Arch. Otolaryngol., 95: 467, 1972.
43. Felson, B.: Less familiar roentgen patterns of pulmonary granulomas. Am. J. Roentgenol. Radium Ther. Nucl. Med., 81: 211, 1959.
44. Ferry, A. P. and Leopold, I. H.: Marginal (ring) corneal ulcer as presenting manifestation of Wegener's granulomatosis. Trans. Am. Acad. Ophthalmol. Otolaryngol., 74: 1276, 1970.
45. Frayer, W. C: The histopathology of perilimbal ulceration in Wegener's granulomatosis. Arch. Ophthalmol., 64: 58, 1960.
46. Fred, H. L., Lynch, E. C., Greenberg, S. D. and Gonzales-Angulo, A: A patient with Wegener's granulomatosis exhibiting unusual clinical and morphological features. Am.J. Med., 37: 311, 1964.
47. Goder, G. and Dölter, J.: Wegenersche granulomatose mit konjunktivalem beginn. Oph-thalmologica, 162: 321, 1971.
48. Godman, G. C. and Churg, J.: Wegener's granulomatosis: pathology and review of the literature. Arch. Path., 58: 533, 1954.
49. Golby, M.: Fertility after renal transplantation. Transplantation, 10: 201, 1970.
50. Goodpasture, E. W.: The significance of certain pulmonary lesions in relation to the etiology of influenza. Am. J. Med. Sci., 158: 863, 1919.
51. Grahne, B. and Zechner, G.: Wegener's granulomatosis. Acta Otolaryngol., 62: 362, 1966.
52. Hansotia, P., Peters, H, Bennett, M. and Brown, R.: Chelation therapy in Wegener's granulomatosis. Treatment with EDTA. Ann. Otol. Rhinol. Laryngol., 78: 388, 1969.
53. Hollander, D. and Manning, R. T.: The use of alkylating agents in the treatment of Wegener's granulomatosis. Ann. Intern. Med., 67: 393, 1967.
54. Israel, H. L. and Patchefsky, A. S.: Wegener's granulomatosis of lung: Diagnosis and treatment. Ann. Intern. Med., 74: 881, 1971.
55. Kalehashi, S, Hammer, J. E., Baer, P. N. and Mclntire, J. A.: Wegener's granulomatosis: report of a case involving the gingiva. Oral Surg, 19: 120, 1965.
56. Kaplan, S. R., Hayslett, J. P. and Calabresi, P.: Treatment of advanced Wegener's granulomatosis with azathioprine and duazomycin. A. N. Engl. J. Med, 278: 239, 1968.
57. Kassel, S. H., Echevarria, R. A. and Guzzo, F. P.: Midline malignant reticulosis: So-called lethal midline granuloma. Cancer 23: 920, 1969.
58. Klinger, H.: Grenzformen der periarteritis nodosa. Frankfurt. Z. Path., 42: 455, 1931.
59. Koffler, D., Sandson, J., Carr, R. and Kunkel, H. G.: Immunologie studies concerning the pulmonary lesions in Goodpasture's syndrome. Am. J. Path., 54: 293, 1969.
60. Kornblum, D. and Fienberg, R.: Roentgen manifestations of necrotizing granulomatosis and angiitis of lungs. Am. J. Roentgenol. Radium Ther. Nucl. Med., 74: 587, 1955.
61. Kraus, Z., Vortel, V., Fingerland, M. S. and Krch, V.: Unusual cutaneous manifestations in Wegener's granulomatosis. Acta Derm. Venereol, 45: 288, 1965.
62. Lamb, A. R.: Periarteritis nodosa. Arch. Intern. Med., 14: 481, 1914.
63. Lerner, R, Glassock, R. J. and Dixon, F. J.: The role of antiglomerular basement membrane antibody in the pathogenesis of human glomerulonephritis. J. Exp. Med., 126: 989, 1967.
64. Levin, D. C.: Pulmonary abnormalities in the necrotizing vasculitides and their rapid response to steroids. Radiology, 97: 521, 1970.
65. Levine, H. and Madden, T. J.: Wegener's granulomatosis: a report of a case. Am. Heart J., 53: 632, 1957.
66. Liebow, A. A. and Carrington, C. B.: The lymphomatoid variant of "limited Wegener's granulomatosis.". Am. J. Path., 55: 78a, 1969.
67. Liebow, A. A., Carrington, C. R. B. and Friedman, P. J.: Lymphomatoid granulomatosis. Hum. Path., 3: 457, 1972.
68. Longauer, Von F., Tokac, M. and Halasova, K.: Uber Schädigung des reizleitungssystems bei Wegenerscher granulomatose. Z. Kreislaufforsch, 58: 412, 1969.
69. Lynch, E. C., Fred, H. L. and Greenberg, S. D.: Pulmonary cavitation in Wegener's granulomatosis. Am. J. Roentgenol. Radium Ther. Nucl. Med., 92: 521, 1964.
70. Manschot, W. A.: The eye in collagen diseases. Adv. Ophthalmol., 11: 1, 1961.
71. McBride, P.: A case of rapid destruction of the nose and face. J. Laryngol. Otol., 12: 64, 1897.
72. McCrea, P. C. and Childers, R. W.: Two unusual cases of giant cell myocarditis associated with mitral stenosis and with Wege-ner's syndrome. Brit. Heart J., 26: 490, 1964.
73. McGregor, M. B. and Sandler, G.: Wegener's granulomatosis. A clinical and radiological survey, Brit, J, Radiol., 37: 430, 1964.
74. Mcllvanie, S. K.: Wegener's granulomatosis: Successful treatment with chlorambucil. J.A.M.A., 197: 90, 1966.
75. McPhaul, J. J., Jr. and Dixon, F. J.: Characterization of human anti-glonrerular basement membrane antibodies cluted from glo-merulonephritic kidneys. J. Clin. Invest., 49: 308, 1970.
76. Merrill, M. D.: Roentgen therapy in Wegener's granulomatosis. Am. J. Roentgenol, 85: 96, 1961.
77. Meyer, J., Foley, J. and Campagna-Pinto. D.: Granulomatous angiitis of the meninges in sarcoidosis. Arch. Neurol. and Psych., 69: 587, 1953.
78. Miller, J. J., Williams, G. F. and Leissring, J. C.: Multiple late complications of therapy with eyclophosphamide, including ovarian destruction. Am. J. Med., 50: 530, 1971.
79. Morgan. A. P. and O'Neill, D.: The oral complications of polyarteritis and giant cell granulomatosis (Wegener's granulomatosis). Oral Surge., 9: 845, 1956.
80. Norton, W. L., Suki. W. and Strand, S.: Combined corticosteroid and azathioprine therapy in two patients with Wegener's granulomatosis. Arch. Intern. Med., 121: 554, 1968.
81. Novack, S. N. and Pearson. C. M.: Cyclophosphamide therapy in Wegener's granulomatosis. N. Engl. J. Med., 284: 938, 1971.
82. O'Duffy, J. D., Scherbel, A. L., Reidbord, H. E. and McCormaek, L. J.: Necrotizing angiitis. I. A clinical review of twenty-seven autopsied cases. Clev. Clin. Q., 32: 87, 1965.
83. Pcermohamed, A. R. and Shafar, J.: Sustained azathioprine-induced remission in Wegener's granulomatosis. Brit. Med., J., 4; 600, 1969.
84. Penn, I. and Starzl, T. E.: Malignant tumors arising de novo in immunosuppressd. organ transplant recipients. Transplantation, 14: 407, 1972.
85. Per-Lee, J. H. and Parsons, R. C.: Vasculitis presenting as otitis media. South. Med. J., 62: 161, 1969.
86. Raitt, J. W.: Wegener's granulomatosis: Treatment with cytotoxic agents and adre-nocorticoids. Ann. Intern. Med.,74: 344. 1971.
87. Reader, W. H. and Goodrich, B. E.: Pulmonary infiltration with eosinophilia (PIE syndrome). Ann. Intern. Med., 36: 1217, 1952.
88. Rebuck, J. W. and Crowley, J. H.: A method of studying leukocyte functions in vivo. Ann. N.Y. Acad. Sci., 59: 757, 1955.
89. Reed. W. B., Jensen, A. K., Konwaler, B. E. and Hunter, D.: The cutaneous manifestations in Wegener'3 granulomatosis. Acta Derm. Venerol., 43: 250, 1963.
90. Rich. A. R. and Gregory, J. E.: The experimental demonstration that pcriarteritis no dosa is a manifestation of hypersensitivity. Bull. Johns Hopkins Hosp., 72: 65, 1943.
91. Roback, S. A., Herdman, R. C., Horger, J. and Good. R. A.: Wegener's granulomatosis in a child. Arn. J. Dis. Child., 118: 608, 1969.
92. Rogers, J. V., Jr. and Roberto, A. E.: Circumscribed pulmonary lesions in periarteritis nodosa and Wegener's granulomatosis. Am. J. Roentgenoh, 76: 88, 1956.
93. SandLer, G. and McGregor. M.: Pulmonary deterioration in Wegener's granulomatosis during steroid therapy. Postgrad. Med. J., 39: 485, 1963.
94. Schwartz, R. S.: Immunosuppressive drug therapy, in Human Transplantation. Edited by Rapaport, F. T. and Dausset, J., Grune and Stratton, Inc. New York, 1968, pp. 440-471.
95. Sevel, D.: Necrogranulomatous keratitis. Am. J. Ophthalmol., 63: 250, 1967.
96. Siltzbach, L. E.: The Kveim test in sarcoidosis. A study of 750 patients. J.A.M.A., 178: 476, 1961.
97. Siltzbach, L. E.: Sarcoidosis-Clinical features and management. Med. Clin. N. Am., 51: 483, 1967.
98. Singh, M. M., Stokes, J. F., Drury, R. A. B. and Walshe, J. M.: Natural history of malignant granuloma of the nose. Lancet, I: 401, 1958.
99. Spear. G. S. and Walker. W. G., Jr.: Lethal midline granuloma (grantiloma gaugraonescens) at autopsy. Bull. Johns Hopkins Hosp., 99: 313, 1956.
100. Stern, G. M., Hoffhrand, A. V. and Urich, H.: The peripheral nerves and skeletal muscles in Wegener's granulomatosis: a clinico-path-oligical siudv of four cases. Brain, 88: 151, 1965.
101. Stewart, J. P.: Progressive lethal granulomatous ulceration of the nose. J. Laryngol. Otol., 48: 657, 1933.
102. Straatsma, B, R.: Ocular manifestations of Wegener's granulomatosis. Arn. J. Ophthal-mol., 44: 789, 1957.
103. Teisberg, P. and Enger. E.: Immunosuppressive therapy in Wegener's granulomatosis. Acta Med. Scand., 187: 7, 1970.
104. Thomas, K.: Laryngeal manifestations of Wegener's granulomatosis. J. Laryngol. Otol., 84: 101, 1970.
105. Tyner. G. S.: Wegener's granulomatosis: A case report. Am.J. Ophtltalmoh. 50: 1203, 1960.
106. Walton, E. W.: Giant-cell granuloma of the respiratory tract (Wegener's granulomatosis). Brit, Med. J., 2: 265, 1958
107. Walton, E. W.: Non-healing granulomala of the nose. J. Larvngol. Otol., 78: 242, 1959.
108. Walton, E. W.: Reticulo-endothelial sarcoma arising in the nose and palate (granuloma gangraenescens). J. Clin. Path., 13: 279, 1960.
109. Wegner. F.: Uber generalisicrie, septische Gefässerkrankungen. Verh. Dtsch. Path. Ges., 29: 202, 1936.
110. Wegner. F.: Uher eine eigenartige rhinogene Granulomatose mit besonderer Beterligung des Arteriensystems und der Nieren. Beitr. Pathol., 102: 36, 1939.
111. Williams, W. J.: A histologic study of the lungs in 52 cases of chronic beryllium disease. Brit. J. Ind. Med., 15: 84, 1958.
112. Yalowitz, P. A., Greene, L. F., Sheps, S. G. and Carlin, M. R.: Wegener's granulomatosis involving the prostate gland: report of a case. J. Urol., 96: 801, 1966.
113. Zeek, P. M.: Periarteritis nodosa: A critical review. Am. J. Clin. Path., 22: 777, 1952.
114. Zeek, P. M.: Periarteritis nodosa and other forms of necrotizing angiitis. N. Engl. J. Med., 248: 764, 1953.
115. Zeek, P. M., Smith, C. C. and Weeter, J. C: Studies on periarteritis nodosa. III. The differentiation between the vascular lesions of periarteritis nodosa and of hypersensitivity. Am. J. Path., 24: 889, 1948.