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Long-term outcomes and prognostic factors for survival of patients with ANCA-associated vasculitis

2023; Oxford University Press; Volume: 38; Issue: 7 Linguagem: Inglês

10.1093/ndt/gfac320

1460-2385

Beatriz Sánchez Álamo, Laura Moi, Ingeborg M. Bajema, Mikkel Faurschou, Oliver Floßmann, Thomas Häuser, Zdenka Hrušková, David Jayne, Raashid Luqmani, Alfred Mahr, Anna Åkesson, Kerstin Westman, Andreas Kronbichler, Neumann Irmgard, Blockmans Daniel, Le Moine Alain, Hruskova Zdenka, Tesar Vladimir, Faurschou Mikkel, Szpirt Wladimir, Ekstrand Agneta, Salmela Anna, Mahr Alfred, Gonzalez Chiappe Solange, Marion Haubitz, Raoul Bergner, Streubert Michaela, Schaier Matthias, S. Wilhelm, Schönenmarck Ulf, De Groot Kirsten, Marinaki Smaragdi, Boletis N. John, Mark A. Little, Gregorini Gina, Vaglio Augusto, Alberici Federico, Renato Alberto Sinico, Garibotto Giacomo, Carta Annalisa, Santostefano Marisa, Brugnano Rachele Maria, Jolanta Dadonienė, Bajema Ingeborg, Berden Annelies, Teng Onno, Cid Maria, Espigol Georgina, Ballarin Jose, García Isabel, Quintana Luis, Fernández Elia Pérez, Fernández Juárez Gema María, Sánchez Álamo Beatriz, Hauser Thomas, Neumann Thomas, Carlo Chizzolini, Balavoine Jean-François, Moi Laura, Bruchfeld Annette, S. Marten, W. Kerstin, A Sukhoveeva Anna, Gunnarsson Iva, J. David, Lorraine Harper, Flossmann Oliver, L. Raashid, M. Steve, L M Goethals Peter, Salama Alan, Dahlsveen Karen, Rosa Joe, Barrett Joe,

Coagulation, Bradykinin, Polyphosphates, and Angioedema

Despite newer treatments with immunosuppressive agents, there still exists a considerable morbidity and mortality risk among patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Since 1994 the European Vasculitis Society (EUVAS) has aimed for an improved outcome for patients with AAV, conducting several prospective randomized controlled trials (RCTs). The aim for the present study was to further evaluate the long-term survival of patients with AAV included in seven RCTs conducted by the EUVAS as well as to identify potential prognostic factors.Long-term follow-up data were collected from questionnaires sent to the principal investigators of the original RCTs (1995-2012): MEPEX, NORAM, CYCAZAREM, CYCLOPS, IMPROVE, RITUXVAS and MYCYC, comprising 848 patients, all newly diagnosed with AAV. Relative survival estimates are presented for the study cohorts. Demographic, clinical and laboratory characteristics at trial entry were studied as potential prognostic factors in multivariable models.A total of 478 (56%) patients had granulomatosis with polyangiitis (GPA) and 370 (44%) had microscopic polyangiitis (MPA) with a mean age at diagnosis of 58 ± 14 years. The median follow-up time was 8 years (interquartile range 2.9-13.6). During the observation period there were 305 deaths and the main causes were infections (26%), cardiovascular disease (14%) and malignancies (13%). When compared with a matched cohort (regarding country, age group and sex) from the background population there were 14.2% more deaths among our cohort of AAV patients at 5 years, 19.9% at 10 years, 28.8% at 15 years and 36.3% at 20 years. The excess mortality occurred in all age groups. The estimated median survival time (from diagnosis) was 17.8 years (95% confidence interval 15.7-20). Among variables measured at baseline, advanced age, male sex, low estimated glomerular filtration rate and low platelet count were identified as predictors of death in a multivariate Cox model.Patients with AAV still have an increased risk of mortality compared with the general population despite newer therapeutic regimens. Treatment complications and organ damage are the main causes of limited survival and infections remain the leading cause of mortality among patients with AAV.