Improved outcome in 445 patients with Wegener's granulomatosis in a German vasculitis center over four decades
| Autor: | Julia U Holle, Martin Heller, Eva Reinhold-Keller, Ute Latza, Wolfgang L. Gross, Bernhard Nölle, Regina Fertmann, Petra Ambrosch |
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| Rok vydání: | 2010 |
| Předmět: |
Adult
Male medicine.medical_specialty Pediatrics Adolescent Prednisolone Immunology Patient Education as Topic Rheumatology Germany Internal medicine medicine Humans Immunology and Allergy Pharmacology (medical) Child Cyclophosphamide Glucocorticoids Aged Aged 80 and over Vascular disease business.industry Cumulative dose Incidence (epidemiology) Remission Induction Granulomatosis with Polyangiitis Middle Aged medicine.disease Confidence interval Surgery Methotrexate Treatment Outcome Antirheumatic Agents Cohort Female business Vasculitis Historical Cohort |
| Zdroj: | Arthritis & Rheumatism. 63:257-266 |
| ISSN: | 0004-3591 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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