Improved outcome in 445 patients with Wegener's granulomatosis in a German vasculitis center over four decades
Top Cited Papers
Open Access
- 22 September 2010
- journal article
- vasculitis
- Published by Wiley in Arthritis & Rheumatism
- Vol. 63 (1), 257-266
- https://doi.org/10.1002/art.27763
Abstract
Objective To determine the long-term outcome in patients with Wegener's granulomatosis (WG) over 4 decades in an academic hospital unit specializing in rheumatology. Methods We included 290 patients, divided them into 2 cohorts, and compared them with the historical cohort of 155 patients. Comparisons were retrospective regarding disease manifestations, therapy, mortality, and incidence of malignancies. The historical cohort (cohort 1) included 155 patients diagnosed between 1966 and 1993, cohort 2 included 123 patients diagnosed between 1994 and 1998, and cohort 3 included 167 patients diagnosed between 1999 and 2002. Results Over time, the interval between first symptoms and diagnosis was reduced by half (from 8 months to 4 months). Organ manifestations were similar in the 3 cohorts, and more than 80% of patients still required cyclophosphamide (CYC); however, the median cumulative dose was reduced significantly (from 67 gm in cohort 1 to 36 gm in cohort 2 and to 24 gm in cohort 3). The standardized mortality ratios (SMRs) declined (from 2.1 in cohort 1 to 1.41 in cohort 2 and to 1.03 in cohort 3), with fewer deaths related to WG and/or therapy (86.4% in cohort 1, 76.9% in cohort 2, 50% in cohort 3), decreasing relapse rates (63.9% in cohort 1, 51.2% in cohort 2, 35.3% in cohort 3), and no increased rate of malignancies. Compared with young females, young males had a considerably higher SMR (8.87 [95% confidence interval 4.05–16.8]) and more frequent renal manifestations (54.4% versus 33.8%). Conclusion Mortality of WG patients declined over the last 4 decades, probably due to improved diagnostic and therapeutic procedures and increased awareness of WG, which led to earlier diagnosis and therapy, reduction in relapse rates, and lower cumulative CYC dose with fewer deaths related to therapy.Keywords
This publication has 34 references indexed in Scilit:
- Improved outcome in Wegener’s granulomatosis and microscopic polyangiitis? A retrospective analysis of 95 cases in two cohortsJournal of Internal Medicine, 2009
- EULAR recommendations for the management of primary small and medium vessel vasculitisAnnals Of The Rheumatic Diseases, 2008
- Wegener's granulomatosis in Finland in 1981–2000: clinical presentation and diagnostic delayScandinavian Journal of Rheumatology, 2008
- Substitution of Methotrexate for Cyclophosphamide in Wegener GranulomatosisMedicine, 2007
- Randomized Trial of Plasma Exchange or High-Dosage Methylprednisolone as Adjunctive Therapy for Severe Renal VasculitisJournal of the American Society of Nephrology, 2007
- Prevalence of Wegener's granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg Strauss syndrome within a defined population in southern SwedenRheumatology, 2007
- Urinary bladder cancer in Wegener's granulomatosis: risks and relation to cyclophosphamideAnnals Of The Rheumatic Diseases, 2004
- Cancer incidence in a population‐based cohort of patients with Wegener's granulomatosisInternational Journal of Cancer, 2002
- Wegener Granulomatosis: An Analysis of 158 PatientsAnnals of Internal Medicine, 1992
- Stage-adapted treatment of Wegener's granulomatosisJournal of Molecular Medicine, 1989