Wegener's granulomatosis: clinical course in 108 patients with renal involvement

Aasarød, Knut; Iversen, Bjarne M.; Hammerstrøm, Jens; Bostad, Leif; Vatten, Lars J.; Jørstad, Størker

doi:10.1093/ndt/15.5.611

Cited by 134 publications

(84 citation statements)

References 24 publications

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“…In a retrospective cohort, 7% of patients developed end stage renal disease at 12 months; increasing to 14% at 5 years and 23% at 10 years 9. In two other studies, end stage renal disease occurred in 19% at 38 months, and 23% at 15 months 3 10. Factors predicting progression to end stage renal disease were as follows.…”

Section: Resultsmentioning

confidence: 87%

“…The remission rate for WG (table 1) ranges from 30% to 93% depending on the definition of remission and remission induction therapy 1 3 12 15 19 – 23. The definition of remission varied from “commencement of clinical improvement”, to “complete absence of disease manifestations for at least 6 months”.…”

Section: Resultsmentioning

confidence: 99%

“…Factors predicting progression to end stage renal disease were as follows. Renal factors: dialysis dependence at diagnosis (RR 3.3 (95% CI 1.3 to 8.8), p = 0.001,3 HR 4.78 (95% CI 1.27 to 17.86), p = 0.02,9 level of evidence = 3). A rise in serum creatinine of 100 μmol/litre (HR 1.35 (95% CI 1.11 to 1.49), p = 0.001,9 level of evidence = 3).…”

Section: Resultsmentioning

confidence: 99%

“…The 5-year survival of treated AAV is over 70%,2 – 5 but relapse and low grade persistent disease result in poor quality survival 4 6 – 8. There is an increased focus on preserving target organ function 3 9 10…”

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confidence: 99%

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Outcomes from studies of antineutrophil cytoplasm antibody associated vasculitis: a systematic review by the European League Against Rheumatism systemic vasculitis task force

Mukhtyar

Floßmann

Hellmich

et al. 2007

Annals of the Rheumatic Diseases

378

250

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Objectives: We undertook a systematic literature review as a background to the European League Against Rheumatism (EULAR) recommendations for conducting clinical trials in anti-neutrophil cytoplasm antibody associated vasculitis (AAV), and to assess the quality of evidence for outcome measures in AAV. Methods: Using a systematic Medline search, we categorised the identified studies according to diagnoses. Factors affecting remission, relapse, renal function and overall survival were identified. Results: A total of 44 papers were reviewed from 502 identified by our search criteria. There was considerable inconsistency in definitions of end points. Remission rates varied from 30% to 93% in Wegener granulomatosis (WG), 75% to 89% in microscopic polyangiitis (MPA) and 81% to 91% in Churg-Strauss syndrome (CSS). The 5-year survival for WG, MPA and CSS was 74-91%, 45-76% and 60-97%. Relapse (variably defined) was common in the first 2 years but the frequency varied: 18% to 60% in WG, 8% in MPA, and 35% in CSS. The rate of renal survival in WG varied from 23% at 15 months to 23% at 120 months. Methods used to assess morbidity varied between studies. Ignoring the variations in definitions of the stage of disease, factors influencing remission, relapse, renal and overall survival included immunosuppressive therapy used, type of organ involvement, presence of ANCA, older age and male gender. Conclusions: Factors influencing remission, relapse, renal and overall survival include the type of immunosuppressive therapy used, pattern of organ involvement, presence of ANCA, older age and male gender. Methodological variations between studies highlight the need for a consensus on terminology and definitions for future conduct of clinical studies in AAV.

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Section: Resultsmentioning

confidence: 87%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

mentioning

confidence: 99%

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Outcomes from studies of antineutrophil cytoplasm antibody associated vasculitis: a systematic review by the European League Against Rheumatism systemic vasculitis task force

Mukhtyar

Floßmann

Hellmich

et al. 2007

Annals of the Rheumatic Diseases

378

250

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“…In PR3-ANCA associated vasculitis, predictive values >50% indicate a relapse within 6–12 months with a >75% increase in the ANCA level [5,6,7], and PR3-ANCA positivity during early follow-up identified patients at increased risk of relapse [8]. Several studies have described clinical features, performed outcome analyses and attempted to determine prognostic markers in WG, the most common PR3-ANCA-associated vasculitis [9,10,11]. …”

Section: Introductionmentioning

confidence: 99%

Clinical Outcome and Prognosis of Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis in Japan

et al. 2010

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Background/Aims: We conducted a broad survey of 99 patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and investigated both prognosis and outcomes. Methods: Clinical data evaluated were age, sex, patient survival, renal survival, serum albumin, serum creatinine, urinary protein, hematuria, C-reactive protein (CRP), ANCA titer, IgG and the Birmingham Vasculitis Activity Score (BVAS). Results: The patient survival rate at 6 months after onset was 84.8%, and that at 2 years after onset was 82.0%. Most deaths were within 6 months of onset. Infection accounted for 9 deaths (60.0%). Infection together with pulmonary involvement of active vasculitis accounted for 2 deaths (13.3%). Organ-specific involvement of active vasculitis alone caused 3 deaths (20.0%). Others died of cardiac events. At 1 and 3 months after onset, BVAS (p < 0.0001, p = 0.002), albumin (p = 0.006, p = 0.0004) and CRP (p = 0.04, p = 0.0002) were also associated with patient death. Conclusion: To improve the prognosis of those with ANCA-associated vasculitis, the intensity of initial treatment should be aimed at disease severity. Employing BVAS improved the ability to evaluate therapeutic responses. Finally, prescription with sulfamethoxazole-trimethoprim during the induction therapy with immunosuppressive agents may be advised.

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Prevalence of Vasculitic Neuropathy in Wegener Granulomatosis

Collins¹,

Periquet²

2002

Archives of Neurology

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Wegener's granulomatosis: clinical course in 108 patients with renal involvement

Abstract: The current treatment of Wegener's granulomatosis does not prevent relapse, development of ESRD and serious treatment-induced infections in a considerable fraction of the patients. Alternative strategies for the management of this disease will be an important objective for further studies.

Cited by 134 publications

References 24 publications

Outcomes from studies of antineutrophil cytoplasm antibody associated vasculitis: a systematic review by the European League Against Rheumatism systemic vasculitis task force

Outcomes from studies of antineutrophil cytoplasm antibody associated vasculitis: a systematic review by the European League Against Rheumatism systemic vasculitis task force

Clinical Outcome and Prognosis of Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis in Japan

Prevalence of Vasculitic Neuropathy in Wegener Granulomatosis

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