Predictors of survival and organ damage in Wegener's granulomatosis

Koldingsnes, Wenche; Nossent, Hans

doi:10.1093/rheumatology/41.5.572

Cited by 145 publications

(138 citation statements)

References 24 publications

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“…Our findings of decreased patient survival in patients with WG or MP with renal involvement is in accordance with those of others (7,12,13,16,(25)(26)(27), although there are difficulties in comparing various studies because of inconsistencies such as great variations in duration of follow-up and the fact that several studies have exclusively included the diagnosis of WG but not MP. We could not detect a worse outcome for the subgroup of patients with WG compared with MP, which may be explained by the fact that in this material, the diagnosis of WG required the presence of a granulomatous disease, histologically verified or strongly suspected by x-ray.…”

Section: Discussionsupporting

confidence: 91%

“…Thus, patients with sinus involvement, epistaxis, or hemoptysis, but without the above-mentioned criteria, received a diagnosis of MP. The dramatic decrease in patient survival during the first year has also been previously described by others (16,17,27). However, to our knowledge, this is the first time that patient survival in ANCA-associated vasculitis with renal involvement has been presented as cumulative relative survival.…”

Section: Discussionsupporting

confidence: 80%

“…We did not find elevated serum creatinine to be a prognostic factor for poor patient survival when we analyzed data by the multiple Cox regression analysis, as has been reported by others (11,16,28,29). A role for serum creatinine at baseline could not be excluded by this study, and interestingly, we found that in the simple Cox regression analysis, elevated serum creatinine seemed to be associated with poorer patient survival.…”

Section: Discussionsupporting

confidence: 79%

“…By means of this approach, we have been able to more accurately evaluate patient survival for the studied disease, which was estimated to be 0.664 for women and 0.648 for men at 10 yr of follow-up. Not surprisingly, older age was a factor in impaired patient survival, as has been noted by others (13,16,25).…”

Section: Discussionsupporting

confidence: 66%

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High Proteinase 3-Anti-Neutrophil Cytoplasmic Antibody (ANCA) Level Measured by the Capture Enzyme-Linked Immunosorbent Assay Method Is Associated with Decreased Patient Survival in ANCA-Associated Vasculitis with Renal Involvement

Westman¹,

Selga

Isberg

et al. 2003

Journal of the American Society of Nephrology

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Abstract. Wegener granulomatosis (WG) and microscopic polyangiitis (MP), diseases associated with antineutrophil cytoplasmic antibodies (ANCA), had an extremely poor prognosis before the introduction of cyclophosphamide and corticosteroids for their treatment. However, there is still reduced patient survival, and some studies have documented severe side effects of the immunosuppressants used. This 10-yr follow-up study assessed 117 consecutive patients with WG or MP with biopsy-confirmed renal involvement. The cumulative relative patient survival was lower: 0.664 for women and 0.648 for men. The causes of death (n ϭ 64) were in most cases registered as associated with the vasculitic disease. Analysis of possible predictive factors for patient survival by multiple Cox regression analysis revealed that a very high level of proteinase 3 (PR3)-ANCA measured by the capture ELISA method, a diagnosis of MP, and older age were factors predicting poorer patient survival. High levels of B-thrombocytes at time of diagnosis were associated with a better prognosis. For patients surviving the first year, remission-sustaining therapy with azathioprine for longer than 12 mo was associated with improved patient survival. Thirty-nine patients developed end-stage renal failure. Elevated serum creatinine at time of diagnosis and a very high level of PR3-ANCA by capture ELISA were factors predicting a higher risk for renal failure during follow-up. The epitope on PR3 assessed by capture ELISA needs to be further analyzed and explored: it seemed to implicate poorer patient and renal survival in WG or MP with renal involvement.

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Section: Discussionsupporting

confidence: 91%

Section: Discussionsupporting

confidence: 80%

Section: Discussionsupporting

confidence: 79%

Section: Discussionsupporting

confidence: 66%

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High Proteinase 3-Anti-Neutrophil Cytoplasmic Antibody (ANCA) Level Measured by the Capture Enzyme-Linked Immunosorbent Assay Method Is Associated with Decreased Patient Survival in ANCA-Associated Vasculitis with Renal Involvement

Westman¹,

Selga

Isberg

et al. 2003

Journal of the American Society of Nephrology

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“…In children, a survival rate between 85 and 100% is reported. [1][2][3] Marmont and VanBekkum were the first to discuss the potential role of allo-SCT for refractory autoimmune diseases. 4 Here, we report the successful treatment of a pediatric WG by allo-SCT following reduced-intensity conditioning.…”

mentioning

confidence: 99%

Long-term remission in pediatric Wegener granulomatosis following allo-SCT after reduced-intensity conditioning

Lawitschka

Peters

Seidel

et al. 2010

Bone Marrow Transplant

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Cutaneous Vasculitis

Levell

Mukhtyar

2016

Rook's Textbook of Dermatology, Ninth Edition

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Overview Vasculitis is usually a multisystem disorder that presents in a myriad of ways. Diagnosis is based on a detailed history and careful examination. Patients may present to different specialties and their care should be led by a multidisciplinary team involving physicians with a specialist interest in vasculitis. Vasculitis can be classified by aetiology or by calibre of the vessel involved. The accepted nomenclature was defined by an international consensus meeting held in 2012; cutaneous vasculitides are considered under the umbrella of ‘single organ vasculitis’, although the classification has been expanded in this chapter. Ill‐defined entities that do not have an identified pathological basis have not been included. It should be recognized that names and classifications will change in the future with greater understanding of the underlying mechanisms of disease. The treatment of primary vasculitis involves immunosuppression. The balance between disease severity and adverse effects of treatment requires expertise and experience in management of these rare conditions. Secondary vasculitis may be due to infection, drugs, malignancy or inflammatory disease. Treating the underlying condition may resolve the vasculitis.

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Predictors of survival and organ damage in Wegener's granulomatosis

Abstract: Treatment with CYC and corticosteroid led to a 10-yr survival rate of 75% in WG but did not prevent severe organ damage. The presence of baseline organ damage was a marker of poor outcome. There was an association between damage and treatment given.

Cited by 145 publications

References 24 publications

High Proteinase 3-Anti-Neutrophil Cytoplasmic Antibody (ANCA) Level Measured by the Capture Enzyme-Linked Immunosorbent Assay Method Is Associated with Decreased Patient Survival in ANCA-Associated Vasculitis with Renal Involvement

High Proteinase 3-Anti-Neutrophil Cytoplasmic Antibody (ANCA) Level Measured by the Capture Enzyme-Linked Immunosorbent Assay Method Is Associated with Decreased Patient Survival in ANCA-Associated Vasculitis with Renal Involvement

Long-term remission in pediatric Wegener granulomatosis following allo-SCT after reduced-intensity conditioning

Cutaneous Vasculitis

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