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/oxfordjournals.ndt.a027949

Cited by 28 publications

(35 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…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%

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

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionsupporting

confidence: 91%

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

View full text Add to dashboard Cite

show abstract

“…Several studies have now shown that, during long-term follow-up, cardiovascular disease is a major cause of mortality in patients with ANCA-associated vasculitis [40][41][42][43][44][45][46][47][48]. Zaenker et al reported that patients with Wegener's granulomatosis had a higher frequency of cardiovascular diseases compared with healthy controls [odds ratio (OR) 6·7] [49].…”

Section: Occurrence Of Accelerated Atherosclerosis In Vasculitismentioning

confidence: 99%

Translational Mini-Review Series on Immunology of Vascular Disease: Accelerated atherosclerosis in vasculitis

Tervaert

2009

Clinical and Experimental Immunology

View full text Add to dashboard Cite

SummaryPremature atherosclerosis has been observed during the course of different systemic inflammatory diseases such as rheumatoid arthritis and sytemic lupus erythematosus. Remarkably, relatively few studies have been published on the occurrence of accelerated atherosclerosis in patients with vasculitis. In giant cell arteritis (GCA), mortality because of ischaemic heart disease is not increased. In addition, intima media thickness (IMT) is lower in patients with GCA than in age-matched controls. In contrast, IMT is increased significantly in Takayasu arteritis, another form of large vessel vasculitis occurring in younger patients. In Takayasu arteritis and in Kawasaki disease, a form of medium-sized vessel vasculitis, accelerated atherosclerosis has been well documented. In small vessel vasculitis because of anti-neutrophil cytoplasmic autoantibodies-associated vasculitis, cardiovascular diseases are a major cause of mortality. IMT measurements reveal conflicting results. During active disease these patients experience acceleration of the atherosclerotic process. However, when inflammation is controlled, these patients have atherosclerotic development as in healthy subjects. Several risk factors, such as diabetes and hypertension, are present more often in patients with vasculitis compared with healthy controls. In addition, steroids may be pro-atherogenic. Most importantly, many patients have impaired renal function, persistent proteinuria and increased levels of C-reactive protein, well-known risk factors for acceleration of atherosclerosis. Enhanced oxidation processes, persistently activated T cells and reduced numbers of regulatory T cells are among the many pathophysiological factors that play a role during acceleration of atherogenesis. Finally, autoantibodies that may be relevant for acceleration of atherosclerosis are found frequently in elevated titres in patients with vasculitis. Because patients have an increased risk for cardiovascular events, vasculitis should be treated with as much care as possible. In addition, treatment should be considered with angiotensin-converting-enzyme inhibitors and/or angiotensin receptor-1 blockers, statins and acetylsalicyl acid. Finally, classical risk factors for cardiovascular disease should be monitored and treated as much as possible.

show abstract

“…Increased mortality has been associated with older age (Ͼ50 years), kidney involvement (with impaired renal function), and pulmonary manifestations at diagnosis in several studies including our own "historical cohort" of 155 WG patients (2). While several studies reported in the 1990s demonstrated an increased mortality of WG patients compared with that in the general population (3)(4)(5)(6), only a slight elevation of the standardized mortality ratios (SMRs) at 5 years (to 1.6) was reported for a recent Swedish population-based cohort of patients with WG and microscopic polyangiitis (MPA) diagnosed between 1978 and 2005 (7), and no increased SMR was reported for women age Ͻ60 years with various vasculitides in a study that assessed a population of patients with disease onset in the 1990s (8).…”

mentioning

confidence: 99%

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

Holle

Gross

Latza

et al. 2010

Arthritis & Rheumatism

251

143

View full text Add to dashboard Cite

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.

show abstract

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

Cited by 28 publications

References 0 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

Translational Mini-Review Series on Immunology of Vascular Disease: Accelerated atherosclerosis in vasculitis

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

Contact Info

Product

Resources

About