Improved outcome in 445 patients with Wegener's granulomatosis in a German vasculitis center over four decades

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 ( 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.

Clinical features and long‐term outcomes of 105 granulomatosis with polyangiitis patients: A single center experience from north India

“…[11][12][13]15 ANCA was negative in 10% of patients in our study, which is similar to that reported from other studies, where ANCA negativity ranged from 10 to 13%. [4][5][6][7][8] Renal involvement was seen in fewer patients in our study population compared to studies from Europe, America and also older studies from India. The more recent Indian study by Kumar et al has also shown that renal involvement is seen in 33% of patients in their cohort.…”

“…GPA seems to affect a relatively younger population in India compared to European and American populations, as the mean age of presentation in our subjects is 40 years which is about a decade earlier than in European and American populations. [4][5][6][7][8] Older Indian studies have noted the mean age of presentation to be between 32 to 42 years, but a recent study documented a mean age of 46 years, which is comparable to that of Western studies. [11][12][13]15 ANCA was negative in 10% of patients in our study, which is similar to that reported from other studies, where ANCA negativity ranged from 10 to 13%.…”

“…2,3 Several large case series regarding GPA patients were published from various countries, with the largest series describing 445 GPA patients from Germany. [4][5][6][7][8] The first report of GPA from India was published in 1985 by Kumar et al followed by descriptions of 11 patients by Bambery et al in 1987. 9,10 Since then various case series have been published from India with the largest series of 45 patients being described by Kumar et al [11][12][13] In the present study, we describe the clinical features, organ involvement, histopathology findings, treatment and outcomes of 105 GPA patients treated at our center, a tertiary care hospital in India.…”

S1 guidelines Diagnostics and treatment of ANCA-associated vasculitis

“…Dementsprechend ist die Akkumulation von Erfahrung in der Diagnostik und Therapie der AAV an die Konzentration dieser Patienten in Zentren gebunden. Mono-und multizentrische Kohorten belegen eine kontinuierliche Verbesserung der Prognose quo ad vitam [93,192,248]. In jüngerer Zeit konnte eine Lebenserwartung, die sich nicht signifikant von der der Allgemeinbevöl-kerung unterschied, bei einem Großteil der AAV-Patienten unter Behandlung durch spezialisierte Zentren erreicht und nachgewiesen werden [93,164,205].…”

“…Mono-und multizentrische Kohorten belegen eine kontinuierliche Verbesserung der Prognose quo ad vitam [93,192,248]. In jüngerer Zeit konnte eine Lebenserwartung, die sich nicht signifikant von der der Allgemeinbevöl-kerung unterschied, bei einem Großteil der AAV-Patienten unter Behandlung durch spezialisierte Zentren erreicht und nachgewiesen werden [93,164,205]. Aus vergleichbaren Zeiträumen und unter ähnlichen (teils jedoch älteren) Therapiestandards gewonnene Daten zeigen ebenso wie historische Daten bei dezentraler Behandlung eine nach wie vor gesteigerte Mortalität gegenüber der Normalbevölkerung [1,53,55,146].…”