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      Early predictors of one-year mortality in patients over 65 presenting with ANCA-associated renal vasculitis: a retrospective, multicentre study

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          Abstract

          Background

          The risk of early death is particularly high in patients over the age of 65 presenting with antineutrophil cytoplasmic antibody (ANCA)-associated renal vasculitis. We hypothesized that by combining disease severity markers, a comorbidity index and serious adverse event reports, we would be able to identify early predictors of one-year mortality in this population.

          Methods

          We performed a multicentre, retrospective study in the nephrology and internal medicine departments of six tertiary hospitals in northern France. A total of 149 patients (median [interquartile range (IQR)] age: 72.7 [68.5–76.8] years) presenting with ANCA-associated vasculitis and renal involvement were included between January 2002 and June 2015. The primary endpoint was the one-year mortality rate.

          Results

          Renal function was severely impaired at presentation (median [IQR] peak serum creatinine (SCr): 337 [211–522] μmol/l), and 45 patients required dialysis. The Five-Factor Score (FFS, scored as + 1 point for each poor prognostic factor (age > 65 years, cardiac symptoms, gastrointestinal involvement, SCr ≥150 μmol/L, and the absence of ear, nose, and throat involvement)) was ≥3 in 120 cases. The one-year mortality rate was 19.5%. Most of the deaths occurred before month 6, and most of these were related to severe infections. In a univariate analysis, age, a high comorbidity index, a performance status of 3 or 4, a lack of co-trimoxazole prophylaxis, early severe infection, and disease activity parameters (such as the albumin level, haemoglobin level, peak SCr level, dialysis status, and high FFS) were significantly associated with one-year mortality. In a multivariable analysis, the best predictors were a high FFS (relative risk (RR) [95% confidence interval (CI)] = 2.57 [1.30–5.09]; p = 0.006) and the occurrence of a severe infection during the first month (RR [95%CI] = 2.74 [1.27–5.92]; p = 0.01).

          Conclusions

          When considering various disease severity markers in over-65 patients with ANCA-associated renal vasculitis, we found that an early, severe infection (which occurred in about a quarter of the patients) is a strong predictor of one-year mortality. A reduction in immunosuppression, the early detection of infections, and co-trimoxazole prophylaxis might help to reduce mortality in this population.

          Electronic supplementary material

          The online version of this article (10.1186/s12882-018-1102-3) contains supplementary material, which is available to authorized users.

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          Most cited references13

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          Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients.

          To retrospectively analyze the clinical symptoms, laboratory findings, and outcomes in patients with microscopic polyangiitis (MPA) who were enrolled in various clinical trials conducted by the French Vasculitis Study Group. A cohort of 85 patients meeting the Chapel Hill criteria for MPA participated in the study. Seventy-one of them were included in prospective therapeutic trials. Eighty-one diagnoses were biopsy proven. In the other patients, diagnosis was based on clinical findings. Forty-seven men and 38 women, with a mean +/- SD age of 56.8 +/- 14.6 years, met the criteria for MPA. Their main clinical symptoms were renal manifestations (78.8%), weight loss (72.9%), skin involvement (62.4%), fever (55.3%), mononeuritis multiplex (57.6%), arthralgias (50.6%), myalgias (48.2%), hypertension (34.1%), lung involvement (24.7%; alveolar hemorrhage 11.8%), and cardiac failure (17.6%). The mean +/- SD serum creatinine level before treatment was 2.59 +/- 2.96 mg/dl; 47 patients had renal insufficiency (serum creatinine > 1.36 mg/dl). Eight patients underwent dialysis at the time of diagnosis, and long-term dialysis was necessary for 10 patients. Antineutrophil cytoplasmic antibodies (ANCA) were present in 38 of 51 patients (74.5%), of whom 33 had a perinuclear staining pattern (pANCA) and 5 had a cytoplasmic pattern. Antibodies to proteinase 3 were present in 4 patients and antibodies to myeloperoxidase were detected in 31, as determined by enzyme-linked immunosorbent assay. Of the 30 patients who underwent renal and celiac angiography, 4 had microaneurysms. Of the 29 patients (34.1%) who had relapses, 8 died during or after the relapse. During followup, 28 of the 85 patients (32.9%) died. The mean +/- SD duration of followup of the group was 69.9 +/- 60.6 months. Deaths were less frequent when patients had been treated with steroids and immunosuppressive drugs (13 patients [24.1%]) than with steroids alone (15 patients [48.4%]) (P < 0.01). The 5-year survival rate was 74%. This study demonstrated that MPA is a multisystemic disease in which renal symptoms are frequent, but the disease is also associated with general symptoms, arthritis, mononeuritis multiplex, and other manifestations that are also seen in various vasculitides. The rarity of abnormal angiogram findings and the high frequency of pANCA are characteristic of MPA. In most cases, the outcome is comparable with those of other systemic vasculitides, but relapses are frequent.
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            Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom.

            To describe the epidemiology of the primary systemic vasculitides (PSV; Wegener's granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, polyarteritis nodosa) in a well-defined population over a 10-year period. An inception cohort of patients from the Norwich Health Authority (NHA) who were >15 years of age and had PSV first diagnosed between January 1, 1988 and December 31, 1997 was collected. Incidence rates were adjusted for age and sex to the 1992 population. The prevalence of PSV in this cohort was estimated on December 31, 1997. Patients were classified according to the American College of Rheumatology 1990 vasculitis criteria and the Chapel Hill Consensus definitions. Eighty-two NHA residents fulfilled the inclusion criteria. There were 47 men and 35 women, with a mean age of 62.9 years (median 65.0 years). The overall annual incidence of PSV among NHA residents was 19.8/million (95% confidence interval [95% CI] 15.8-24.6). The point prevalence on December 31, 1997 was 144.5/million (95% CI 110.4-185.3). PSV was more common in males (23.5/million; 95% CI 17.3-31.3) than females (16.4/million; 95% CI 11.4-22.8). The age- and sex-specific incidence showed a clear increase with age, with an overall peak in the 65-74 year age group (60.1/million). In our study population, the annual incidence of PSV is slowly increasing with time and the incidence is greatest in the elderly.
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              Predictors of treatment outcomes in ANCA-associated vasculitis with severe kidney failure.

              In ANCA-associated GN, severe renal dysfunction portends a poor prognosis for renal recovery and patient survival. This study evaluated the prognostic factors affecting renal and patient outcomes in patients presenting with severe kidney failure to guide immunosuppressive therapy.
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                Author and article information

                Contributors
                +33-322-456-411 , titeca.dimitri@chu-amiens.fr
                Journal
                BMC Nephrol
                BMC Nephrol
                BMC Nephrology
                BioMed Central (London )
                1471-2369
                9 November 2018
                9 November 2018
                2018
                : 19
                : 317
                Affiliations
                [1 ]ISNI 0000 0004 0593 702X, GRID grid.134996.0, Department of Nephrology, Dialysis and Transplantation, , Amiens University Hospital, ; F-80054 Amiens, France
                [2 ]ISNI 0000 0004 0472 0160, GRID grid.411149.8, Department of Nephrology, , Caen University Hospital, ; Caen, France
                [3 ]Registre de Dialyse Péritonéale de Langue Française, Pontoise, France
                [4 ]GRID grid.41724.34, Department of Nephrology, , Rouen University Hospital, ; Rouen, France
                [5 ]INSERM, U1096 Rouen, France
                [6 ]ISNI 0000 0001 2186 1211, GRID grid.4461.7, University of Lille, ; U995 Lille, France
                [7 ]Lille Inflammation Research International Center (LIRIC), Lille, France
                [8 ]GRID grid.457380.d, Inserm, ; U995 Lille, France
                [9 ]ISNI 0000 0004 0471 8845, GRID grid.410463.4, Département de Médecine Interne et Immunologie Clinique, , CHU Lille, ; Lille, France
                [10 ]Centre national de Référence Maladies Systémiques et Auto-immunes Rares (Sclérodermie Systémique), Lille, France
                [11 ]Department of Nephrology and Internal Medicine, Valenciennes General Hospital, Valenciennes, France
                [12 ]Department of Nephrology, Duchenne Hospital, Boulogne-sur-Mer, France
                [13 ]ISNI 0000 0004 0471 8845, GRID grid.410463.4, Department of Nephrology, Calmette Hospital, , Lille University Hospital, ; Lille, France
                [14 ]Department of Internal Medicine, Caen, France
                [15 ]ISNI 0000 0001 2186 4076, GRID grid.412043.0, Normandie Univ, UNICAEN, INSERM, COMETE, ; Caen, France
                [16 ]ISNI 0000 0004 0593 702X, GRID grid.134996.0, Clinical Research and Innovation Directorate, , Amiens University Hospital, ; Amiens, France
                Author information
                http://orcid.org/0000-0003-2514-4193
                Article
                1102
                10.1186/s12882-018-1102-3
                6234782
                30413153
                0bca7ace-4e93-4b49-a4ac-03f901e8787f
                © The Author(s). 2018

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                History
                : 28 December 2017
                : 12 October 2018
                Categories
                Research Article
                Custom metadata
                © The Author(s) 2018

                Nephrology
                anca,elderly,glomerulonephritis,infection,mortality
                Nephrology
                anca, elderly, glomerulonephritis, infection, mortality

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