Review
15 March 1992

Wegener Granulomatosis: An Analysis of 158 Patients

Publication: Annals of Internal Medicine
Volume 116, Number 6

Abstract

Objective: To prospectively study the clinical features, pathophysiology, treatment, and prognosis of Wegener granulomatosis.
Design: Of the 180 patients with Wegener granulomatosis referred to the National Institute of Allergy and Infectious Diseases during the past 24 years, 158 have been followed for 6 months to 24 years (a total of 1229 patient-years).
Measurements: Characteristics of clinical presentation, surgical pathology, course of illness, laboratory and radiographic findings, and the results of medical and surgical treatment have been recorded in a computer-based information retrieval system.
Setting: The Warren Magnuson Clinical Center of the National Institutes of Health.
Main Results: Men and women were equally represented; 97% of patients were white, and 85% were more than 19 years of age. The mean period of follow-up was 8 years. One hundred and thirty-three patients (84%) received "standard" therapy with daily low-dose cyclophosphamide and glucocorticoids. Eight (5.0%) received only low-dose cyclophosphamide. Six (4.0%) never received cyclophosphamide and were treated with other cytotoxic agents and glucocorticoids. Ten patients (6.0%) were treated with only glucocorticoids. Ninety-one percent of patients experienced marked improvement, and 75% achieved complete remission. Fifty percent of remissions were associated with one or more relapses. Of 99 patients followed for > 5 years, 44% had remissions of > 5 years duration. Thirteen percent of patients died of Wegener granulomatosis, treatment-related causes, or both. Almost all patients had serious morbidity from irreversible features of their disease (86%) or side effects of treatment (42%).
Conclusions: The course of Wegener granulomatosis has been dramatically improved by daily treatment with cyclophosphamide and glucocorticoids. Nonetheless, disease- and treatment-related morbidity is often profound. Alternative forms of therapy have not yet achieved the high rates of remission induction and successful maintenance that have been reported with daily cyclophosphamide treatment. Despite continued therapeutic success with cyclophosphamide, our longterm follow-up of patients with Wegener granulomatosis has led to increasing concerns about toxicity resulting from prolonged cyclophosphamide therapy and has encouraged investigation of other therapeutic regimens. Annals of Internal Medicine. 1992;116:488-498.

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Information & Authors

Information

Published In

cover image Annals of Internal Medicine
Annals of Internal Medicine
Volume 116Number 615 March 1992
Pages: 488 - 498

History

Published in issue: 15 March 1992
Published online: 1 December 2008

Keywords

Authors

Affiliations

Randi Y. Leavitt, MD, PhD
Claire W. Hallahan, MS
Robert S. Lebovics, MD
William D. Travis, MD
From the National Institutes of Health, Bethesda, Maryland. For current author addresses, see end of text.

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Gary S. Hoffman, Gail S. Kerr, Randi Y. Leavitt, et al. Wegener Granulomatosis: An Analysis of 158 Patients. Ann Intern Med.1992;116:488-498. doi:10.7326/0003-4819-116-6-488

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