title: Clinical characteristics and outcome of Spanish patients with ANCA-associated vasculitides: Impact of the vasculitis type, ANCA specificity, and treatment on mortality and morbidity.
abstract: The 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.
authors.affiliation: Hospital Valle Hebrón, Barcelona Hospital Ramón y Cajal, Madrid Mútua Terrassa, Barcelona Hospital Central de Asturias Hospital Virgen del Rocío, Sevilla Hospital Miguel Servet, Zaragoza Hospital La Paz, Madrid Hospital Bellvitge Hospital Parc Tauli Sabadell, Barcelona Hospital Marina Baixa, Villajoyosa, Alicante Hospital Cabueñes, Asturias Hospital Virgen de las Nieves Hospital Clínico San Cecilio, Granada Hospital Fuenlabrada, Madrid Hospital La Ribera, Alzira, Valencia Clínica de Navarra Hospital Infanta Leonor, Madrid Hospital Joan XXIII, Tarragona Centro Hospitalário Vigo Hospital Ourense, Galicia, Spain.
authors.forename: Roser
authors.initials: R
authors.lastname: Solans-Laqué
authors.forename: Guadalupe
authors.initials: G
authors.lastname: Fraile
authors.forename: Monica
authors.initials: M
authors.lastname: Rodriguez-Carballeira
authors.forename: Luis
authors.initials: L
authors.lastname: Caminal
authors.forename: Maria J
authors.initials: MJ
authors.lastname: Castillo
authors.forename: Ferran
authors.initials: F
authors.lastname: Martínez-Valle
authors.forename: Luis
authors.initials: L
authors.lastname: Sáez
authors.forename: Juan J
authors.initials: JJ
authors.lastname: Rios
authors.forename: Xavier
authors.initials: X
authors.lastname: Solanich
authors.forename: Joaquim
authors.initials: J
authors.lastname: Oristrell
authors.forename: Francisco
authors.initials: F
authors.lastname: Pasquau
authors.forename: Eva
authors.initials: E
authors.lastname: Fonseca
authors.forename: Monica
authors.initials: M
authors.lastname: Zamora
authors.forename: Jose L
authors.initials: JL
authors.lastname: Callejas
authors.forename: Begoña
authors.initials: B
authors.lastname: Frutos
authors.forename: Monica
authors.initials: M
authors.lastname: Abdilla
authors.forename: Patricia
authors.initials: P
authors.lastname: Fanlo
authors.forename: Isabel
authors.initials: I
authors.lastname: García-Sánchez
authors.forename: Miguel
authors.initials: M
authors.lastname: López-Dupla
authors.forename: Bernardo
authors.initials: B
authors.lastname: Sopeña
authors.forename: Almudena
authors.initials: A
authors.lastname: Pérez-Iglesias
authors.forename: Josep A
authors.initials: JA
authors.lastname: Bosch
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000188:drug therapy* / Q000401:mortality* / Q000503:physiopathology; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000378:metabolism*; D015897:Comorbidity; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012008:Recurrence; D012189:Retrospective Studies; D013030:Spain / Q000453:epidemiology; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic