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 greater than 5 years, 44% had remissions of greater than 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:
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Authors | G S Hoffman, G S Kerr, R Y Leavitt, C W Hallahan, R S Lebovics, W D Travis, M Rottem, A S Fauci |
Journal | Annals of internal medicine
(Ann Intern Med)
Vol. 116
Issue 6
Pg. 488-98
(Mar 15 1992)
ISSN: 0003-4819 [Print] United States |
PMID | 1739240
(Publication Type: Journal Article)
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Chemical References |
- Cyclophosphamide
- Prednisone
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Topics |
- Adolescent
- Adult
- Aged
- Biopsy
- Child
- Cyclophosphamide
(adverse effects, therapeutic use)
- Drug Therapy, Combination
- Female
- Follow-Up Studies
- Granulomatosis with Polyangiitis
(complications, diagnosis, drug therapy, mortality)
- Humans
- Male
- Middle Aged
- Morbidity
- Opportunistic Infections
(epidemiology)
- Prednisone
(adverse effects, therapeutic use)
- Recurrence
- Remission Induction
- Treatment Outcome
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