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dc.contributorVall d'Hebron Barcelona Hospital Campus
dc.contributor.authorSolans Laque, Roser
dc.contributor.authorFraile, Guadalupe
dc.contributor.authorRodriguez-Carballeira, Monica
dc.contributor.authorCaminal, Luis
dc.contributor.authorCastillo, Maria J
dc.contributor.authorMartínez Valle, Fernando
dc.contributor.authorZamora Villafaina, Monica
dc.contributor.authorBosch Gil, José Ángel
dc.date.accessioned2019-03-22T07:12:00Z
dc.date.available2019-03-22T07:12:00Z
dc.date.issued2017-02
dc.identifier.citationSolans-Laqué R, Fraile G, Rodriguez-Carballeira M, Caminal L, Castillo MJ, Martínez-Valle F, et al. Clinical characteristics and outcome of Spanish patients with ANCA-associated vasculitides: Impact of the vasculitis type, ANCA specificity, and treatment on mortality and morbidity. Medicine (Baltimore). 2017;96(8):e6083.
dc.identifier.issn0025-7974
dc.identifier.urihttp://hdl.handle.net/11351/3856
dc.descriptionSpain; ANCA; Vasculitides
dc.description.abstractThe 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 high-cumulated 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.
dc.language.isoeng
dc.publisherLippincott, Williams & Wilkins
dc.relation.ispartofseriesMedicine;96(8)
dc.rightsAttribution-NonCommercial-NoDerivatives 4.0 International
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/
dc.sourceScientia
dc.subjectComorbiditat
dc.subjectVasculitis - Espanya
dc.subject.meshAnti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
dc.subject.mesh/mortality
dc.subject.mesh/physiopathology
dc.subject.meshComorbidity
dc.subject.meshSpain
dc.subject.mesh/epidemiology
dc.titleClinical characteristics and outcome of Spanish patients with ANCA-associated vasculitides: Impact of the vasculitis type, ANCA specificity, and treatment on mortality and morbidity
dc.typeinfo:eu-repo/semantics/article
dc.identifier.doi10.1097/MD.0000000000006083
dc.subject.decsvasculitis asociada a anticuerpos anticitoplasma de neutrófilos
dc.subject.decs/mortalidad
dc.subject.decs/fisiopatología
dc.subject.decscomorbilidad
dc.subject.decsEspaña
dc.subject.decs/epidemiología
dc.relation.publishversionhttps://journals.lww.com/md-journal/fulltext/2017/02240/Clinical_characteristics_and_outcome_of_Spanish.9.aspx#pdf-link
dc.type.versioninfo:eu-repo/semantics/publishedVersion
dc.audienceProfessionals
dc.contributor.organismesInstitut Català de la Salut
dc.contributor.authoraffiliation[Solans-Laqué R] Hospital Universitari Vall d'Hebron, Barcelona, Spain. [Fraile G] Hospital Ramón y Cajal, Madrid, Spain. [Rodriguez-Carballeira M] Mútua Terrassa, Barcelona, Spain. [Caminal L] Hospital Central de Asturias, Oviedo, Spain. [Castillo MJ] Hospital Virgen del Rocío, Sevilla, Spain. [Martínez-Valle F, Zamora M, Bosch JA] Hospital Universitari Vall d'Hebron, Barcelona, Spain.
dc.identifier.pmid28225490
dc.identifier.wosWOS:000395794000008
dc.rights.accessrightsinfo:eu-repo/semantics/openAccess


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