000063237 001__ 63237 000063237 005__ 20190709135502.0 000063237 0247_ $$2doi$$a10.1097/MD.0000000000006083 000063237 0248_ $$2sideral$$a98612 000063237 037__ $$aART-2017-98612 000063237 041__ $$aeng 000063237 100__ $$aSolans-Laqué, R. 000063237 245__ $$aClinical characteristics and outcome of Spanish patients with ANCA-associated vasculitides Impact of the vasculitis type, ANCA specificity, and treatment on mortality and morbidity 000063237 260__ $$c2017 000063237 5060_ $$aAccess copy available to the general public$$fUnrestricted 000063237 5203_ $$aThe aim of this study was to describe the clinical characteristics of ANCA-associated vasculitides (AAV) at presentation, in a wide cohort of Spanish patients, and to analyze the impact of the vasculitis type, ANCA specificity, prognostic factors, and treatments administered at diagnosis, in the outcome. A total of 450 patients diagnosed between January 1990 and January 2014 in 20 Hospitals from Spain were included. Altogether, 40.9% had granulomatosis with polyangiitis (GPA), 37.1% microscopic polyangiitis (MPA), and 22% eosinophilic granulomatosis with polyangiitis (EGPA). The mean age at diagnosis was 55.6±17.3 years, patients with MPA being significantly older (P<0.001). Fever, arthralgia, weight loss, respiratory, and ear-nose-throat (ENT) symptoms, were the most common at disease onset. ANCAs tested positive in 86.4% of cases: 36.2% C-ANCA-PR3 and 50.2% P-ANCA-MPO. P-ANCA-MPO was significantly associated with an increased risk for renal disease (OR 2.6, P<0.001) and alveolar hemorrhage (OR 2, P=0.010), while C-ANCA-PR3 was significantly associated with an increased risk for ENT (OR 3.4, P<0.001) and ocular involvement (OR 2.3, P=0.002). All patients received corticosteroids (CS) and 74.9% cyclophosphamide (CYC). The median follow-up was 82 months (IQR 100.4). Over this period 39.9% of patients suffered bacterial infections and 14.6% opportunistic infections, both being most prevalent in patients with highcumulated doses of CYC and CS (P<0.001). Relapses were recorded in 36.4% of cases with a mean rate of 2.5±2.3, and were more frequent in patients with C-ANCA-PR3 (P=0.012). The initial disease severity was significantly associated with mortality but not with the occurrence of relapses. One hundred twenty-nine (28.7%) patients (74 MPA, 41 GPA, 14 EGPA) died. The mean survival was 58 months (IQR 105) and was significantly lower for patients with MPA (P<0.001). Factors independently related to death were renal involvement (P=0.010), cardiac failure (P=0.029) and age over 65 years old (P<0.001) at disease onset, and bacterial infections (P<0.001). An improved outcome with significant decrease in mortality and treatment-related morbidity was observed in patients diagnosed after 2000, and was related to the implementation of less toxic regimens adapted to the disease activity and stage, and a drastic reduction in the cumulated CYC and CS dose. 000063237 540__ $$9info:eu-repo/semantics/openAccess$$aby-nc-nd$$uhttp://creativecommons.org/licenses/by-nc-nd/3.0/es/ 000063237 590__ $$a2.028$$b2017 000063237 591__ $$aMEDICINE, GENERAL & INTERNAL$$b56 / 154 = 0.364$$c2017$$dQ2$$eT2 000063237 592__ $$a0.168$$b2017 000063237 593__ $$aMedicine (miscellaneous)$$c2017$$dQ3 000063237 655_4 $$ainfo:eu-repo/semantics/article$$vinfo:eu-repo/semantics/publishedVersion 000063237 700__ $$aFraile, G. 000063237 700__ $$aRodriguez-Carballeira, M. 000063237 700__ $$aCaminal, L. 000063237 700__ $$aCastillo, M. J. 000063237 700__ $$aMartínez-Valle, F. 000063237 700__ $$0(orcid)0000-0002-9868-6498$$aSáez, L.$$uUniversidad de Zaragoza 000063237 700__ $$aRios, J. J. 000063237 700__ $$aSolanich, X. 000063237 700__ $$aOristrell, J. 000063237 700__ $$aPasquau, F. 000063237 700__ $$aFonseca, E. 000063237 700__ $$aZamora, M. 000063237 700__ $$aCallejas, J. L. 000063237 700__ $$aFrutos, B. 000063237 700__ $$aAbdilla, M. 000063237 700__ $$aFanlo, P. 000063237 700__ $$aGarcía-Sánchez, I. 000063237 700__ $$aLópez-Dupla, M. 000063237 700__ $$aSopeña, B. 000063237 700__ $$aPérez-Iglesias, A. 000063237 700__ $$aBosch, J. A. 000063237 7102_ $$11007$$2610$$aUniversidad de Zaragoza$$bDpto. Medicina, Psiqu. y Derm.$$cArea Medicina 000063237 773__ $$g96, 8 (2017), e6083$$pMedicine (Baltim.)$$tMedicine$$x0025-7974 000063237 8564_ $$s346311$$uhttps://zaguan.unizar.es/record/63237/files/texto_completo.pdf$$yVersión publicada 000063237 8564_ $$s122323$$uhttps://zaguan.unizar.es/record/63237/files/texto_completo.jpg?subformat=icon$$xicon$$yVersión publicada 000063237 909CO $$ooai:zaguan.unizar.es:63237$$particulos$$pdriver 000063237 951__ $$a2019-07-09-11:46:50 000063237 980__ $$aARTICLE