BACKGROUND AND OBJECTIVES: ANCA-associated vasculitis is commonly found in elderly patients, but there are few data concerning outcome and treatment in the highest age groups. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Consecutive patients (N=151) presenting between 1997 and 2009 were retrospectively included from local registries in six centers in Sweden, the United Kingdom, and the Czech Republic if diagnosed with microscopic polyangiitis or granulomatosis with polyangiitis at age ≥75 years during the study period. Patients were followed until 2 years from diagnosis or death. Data on survival and renal function were analyzed with respect to age, sex, ANCA specificity, renal function, C-reactive protein, comorbidities, and Birmingham Vasculitis Activity Score at diagnosis as well as treatment during the first month. RESULTS: Median follow-up was 730 days (interquartile range, 244-730). Overall 1-year survival was 71.5% and 2-year survival was 64.6%. Older age, higher creatinine, and lower Birmingham Vasculitis Activity Score were associated with higher mortality in multivariable analysis. Patients who were not treated with standard immunosuppressive therapy had significantly worse survival. Renal survival was 74.8% at 1 year. No new cases of ESRD occurred during the second year. High creatinine at diagnosis was the only significant predictor of renal survival in multivariable analysis. CONCLUSIONS: ANCA-associated vasculitis is a disease with substantial mortality and morbidity among elderly patients. This study showed a better prognosis for those who received immunosuppressive treatment and those who were diagnosed before having developed advanced renal insufficiency.

Department of Clinical Sciences Section of Rheumatology Lund University Lund Sweden; Vasculitis and Lupus Clinic Addenbrooke's Hospital Cambridge United Kingdom;

Department of Medicine Imperial College London London United Kingdom;

Department of Nephrology 1st Faculty of Medicine Charles University Prague and General University Hospital Prague Czech Republic;

Department of Nephrology Lund University Skåne University Hospital Lund and Malmö Sweden

Department of Renal Medicine Karolinska University Hospital and Department of Clinical Sciences Intervention and Technology Karolinska Institute Stockholm Sweden; and

Departments of Nephrology and Medical and Health Sciences and

Rheumatology and Clinical and Experimental Medicine Linköping University Linköping Sweden;

University College London Centre for Nephrology Royal Free Hospital London United Kingdom;

Clin J Am Soc Nephrol. 2015 Jul 7;10(7):1110-3. doi: 10.2215/CJN.05350515. PubMed

Zobrazit více v PubMed

Jennette JC, Falk RJ, Hu P, Xiao H: Pathogenesis of antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitis. Annu Rev Pathol 8: 139–160, 2013 PubMed PMC

Booth AD, Almond MK, Burns A, Ellis P, Gaskin G, Neild GH, Plaisance M, Pusey CD, Jayne DR, Pan-Thames Renal Research Group : Outcome of ANCA-associated renal vasculitis: A 5-year retrospective study. Am J Kidney Dis 41: 776–784, 2003 PubMed

Mohammad AJ, Jacobsson LT, Westman KW, Sturfelt G, Segelmark M: Incidence and survival rates in Wegener’s granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and polyarteritis nodosa. Rheumatology (Oxford) 48: 1560–1565, 2009 PubMed

Ntatsaki E, Watts RA, Scott DG: Epidemiology of ANCA-associated vasculitis. Rheum Dis Clin North Am 36: 447–461, 2010 PubMed

Harper L, Savage CO: ANCA-associated renal vasculitis at the end of the twentieth century—a disease of older patients. Rheumatology (Oxford) 44: 495–501, 2005 PubMed

Chen M, Yu F, Zhang Y, Zhao MH: Antineutrophil cytoplasmic autoantibody-associated vasculitis in older patients. Medicine (Baltimore) 87: 203–209, 2008 PubMed

Little MA, Nightingale P, Verburgh CA, Hauser T, De Groot K, Savage C, Jayne D, Harper L, European Vasculitis Study (EUVAS) Group : Early mortality in systemic vasculitis: Relative contribution of adverse events and active vasculitis. Ann Rheum Dis 69: 1036–1043, 2010 PubMed

Harper L, Morgan MD, Walsh M, Hoglund P, Westman K, Flossmann O, Tesar V, Vanhille P, de Groot K, Luqmani R, Flores-Suarez LF, Watts R, Pusey C, Bruchfeld A, Rasmussen N, Blockmans D, Savage CO, Jayne D, EUVAS investigators : Pulse versus daily oral cyclophosphamide for induction of remission in ANCA-associated vasculitis: Long-term follow-up. Ann Rheum Dis 71: 955–960, 2012 PubMed

Mukhtyar C, Guillevin L, Cid MC, Dasgupta B, de Groot K, Gross W, Hauser T, Hellmich B, Jayne D, Kallenberg CG, Merkel PA, Raspe H, Salvarani C, Scott DG, Stegeman C, Watts R, Westman K, Witter J, Yazici H, Luqmani R, European Vasculitis Study Group : EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis 68: 310–317, 2009 PubMed

Tarzi RM, Pusey CD: Vasculitis: Risks and rewards of treating elderly patients with vasculitis. Nat Rev Nephrol 7: 253–255, 2011 PubMed

Hoganson DD, From AM, Michet CJ: ANCA vasculitis in the elderly. J Clin Rheumatol 14: 78–81, 2008 PubMed

Bomback AS, Appel GB, Radhakrishnan J, Shirazian S, Herlitz LC, Stokes B, D’Agati VD, Markowitz GS: ANCA-associated glomerulonephritis in the very elderly. Kidney Int 79: 757–764, 2011 PubMed

Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, Mahr A, Segelmark M, Cohen-Tervaert JW, Scott D: Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 66: 222–227, 2007 PubMed PMC

Eriksson P, Jacobsson L, Lindell A, Nilsson JA, Skogh T: Improved outcome in Wegener’s granulomatosis and microscopic polyangiitis? A retrospective analysis of 95 cases in two cohorts. J Intern Med 265: 496–506, 2009 PubMed

Flossmann O, Bacon P, de Groot K, Jayne D, Rasmussen N, Seo P, Westman K, Luqmani R: Development of comprehensive disease assessment in systemic vasculitis. Ann Rheum Dis 66: 283–292, 2007 PubMed PMC

Davies SJ, Russell L, Bryan J, Phillips L, Russell GI: Comorbidity, urea kinetics, and appetite in continuous ambulatory peritoneal dialysis patients: Their interrelationship and prediction of survival. Am J Kidney Dis 26: 353–361, 1995 PubMed

Westman KW, Selga D, Isberg PE, Bladström A, Olsson H: 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. J Am Soc Nephrol 14: 2926–2933, 2003 PubMed

Flossmann O, Berden A, de Groot K, Hagen C, Harper L, Heijl C, Höglund P, Jayne D, Luqmani R, Mahr A, Mukhtyar C, Pusey C, Rasmussen N, Stegeman C, Walsh M, Westman K, European Vasculitis Study Group : Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis 70: 488–494, 2011 PubMed

Mukhtyar C, Flossmann O, Hellmich B, Bacon P, Cid M, Cohen-Tervaert JW, Gross WL, Guillevin L, Jayne D, Mahr A, Merkel PA, Raspe H, Scott D, Witter J, Yazici H, Luqmani RA, European Vasculitis Study Group (EUVAS) : Outcomes from studies of antineutrophil cytoplasm antibody associated vasculitis: A systematic review by the European League Against Rheumatism systemic vasculitis task force. Ann Rheum Dis 67: 1004–1010, 2008 PubMed

Slot MC, Tervaert JW, Franssen CF, Stegeman CA: Renal survival and prognostic factors in patients with PR3-ANCA associated vasculitis with renal involvement. Kidney Int 63: 670–677, 2003 PubMed

Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, Kallenberg CG, St Clair EW, Turkiewicz A, Tchao NK, Webber L, Ding L, Sejismundo LP, Mieras K, Weitzenkamp D, Ikle D, Seyfert-Margolis V, Mueller M, Brunetta P, Allen NB, Fervenza FC, Geetha D, Keogh KA, Kissin EY, Monach PA, Peikert T, Stegeman C, Ytterberg SR, Specks U, RAVE-ITN Research Group : Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 363: 221–232, 2010 PubMed PMC

Silva F, Seo P, Schroeder DR, Stone JH, Merkel PA, Hoffman GS, Spiera R, Sebastian JK, Davis JC, Jr, St Clair EW, Allen NB, McCune WJ, Ytterberg SR, Specks U, Wegener’s Granulomatosis Etanercept Trial Research Group : Solid malignancies among etanercept-treated patients with granulomatosis with polyangiitis (Wegener’s): Long-term followup of a multicenter longitudinal cohort. Arthritis Rheum 63: 2495–2503, 2011 PubMed PMC

Organization for Economic Cooperation and Development: Life expectancy at birth, total population. Health: Key tables from OECD 2010. No. 11, 2010. Available at: http://dx.doi.org/10.1787/20758480-2010-table8. Accessed May 14, 2015 DOI

Mahr A, Katsahian S, Varet H, Guillevin L, Hagen EC, Höglund P, Merkel PA, Pagnoux C, Rasmussen N, Westman K, Jayne DR, French Vasculitis Study Group (FVSG) and the European Vasculitis Society (EUVAS) : Revisiting the classification of clinical phenotypes of anti-neutrophil cytoplasmic antibody-associated vasculitis: A cluster analysis. Ann Rheum Dis 72: 1003–1010, 2013 PubMed

de Joode AA, Sanders JS, Stegeman CA: Renal survival in proteinase 3 and myeloperoxidase ANCA-associated systemic vasculitis. Clin J Am Soc Nephrol 8: 1709–1717, 2013 PubMed PMC

Krafcik SS, Covin RB, Lynch JP, 3rd, Sitrin RG: Wegener’s granulomatosis in the elderly. Chest 109: 430–437, 1996 PubMed

Lyons PA, Rayner TF, Trivedi S, Holle JU, Watts RA, Jayne DR, Baslund B, Brenchley P, Bruchfeld A, Chaudhry AN, Cohen Tervaert JW, Deloukas P, Feighery C, Gross WL, Guillevin L, Gunnarsson I, Harper L, Hrušková Z, Little MA, Martorana D, Neumann T, Ohlsson S, Padmanabhan S, Pusey CD, Salama AD, Sanders JS, Savage CO, Segelmark M, Stegeman CA, Tesař V, Vaglio A, Wieczorek S, Wilde B, Zwerina J, Rees AJ, Clayton DG, Smith KG: Genetically distinct subsets within ANCA-associated vasculitis. N Engl J Med 367: 214–223, 2012 PubMed PMC

Kaplan-Pavlovcic S, Cerk K, Kveder R, Lindic J, Vizjak A: Clinical prognostic factors of renal outcome in anti-neutrophil cytoplasmic autoantibody (ANCA)-associated glomerulonephritis in elderly patients. Nephrol Dial Transplant 18[Suppl 5]: v5–v7, 2003 PubMed

Hogan SL, Nachman PH, Wilkman AS, Jennette JC, Falk RJ: Prognostic markers in patients with antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis. J Am Soc Nephrol 7: 23–32, 1996 PubMed

Jayne DR, Gaskin G, Rasmussen N, Abramowicz D, Ferrario F, Guillevin L, Mirapeix E, Savage CO, Sinico RA, Stegeman CA, Westman KW, van der Woude FJ, de Lind van Wijngaarden RA, Pusey CD, European Vasculitis Study Group : Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol 18: 2180–2188, 2007 PubMed

Data quality and patient characteristics in European ANCA-associated vasculitis registries: data retrieval by federated querying