RT Journal Article
T1 Clinical characteristics and outcome of Spanish patients with ANCA-associated vasculitides: Impact of the vasculitis type, ANCA specificity, and treatment on mortality and morbidity
A1 Solans-Laqué, Roser
A1 Fraile, Guadalupe
A1 Rodriguez-Carballeira, Monica
A1 Caminal, Luis
A1 Castillo, Maria J
A1 Martínez-Valle, Ferran
A1 Sáez, Luis
A1 Rios, Juan J
A1 Solanich, Xavier
A1 Oristrell, Joaquim
A1 Pasquau, Francisco
A1 Fonseca, Eva
A1 Zamora, Monica
A1 Callejas, Jose L
A1 Frutos, Begoña
A1 Abdilla, Monica
A1 Fanlo, Patricia
A1 García-Sánchez, Isabel
A1 López-Dupla, Miguel
A1 Sopeña, Bernardo
A1 Pérez-Iglesias, Almudena
A1 Bosch, Josep A
K1 ANCA-associated vasculitides
K1 Eosinophilic granulomatosis with polyangiitis
K1 Granulomatosis with polyangiitis
K1 Infections
K1 Microscopic polyangiitis
K1 Mortality predictors
K1 Outome
K1 Spanish people
K1 Resultado del tratamiento
K1 Adulto joven
K1 Adolescente
K1 Adulto
K1 Anciano
K1 Anciano de 80 o más años
K1 Vasculitis asociada a anticuerpos citoplasmáticos antineutrófilos
K1 Anticuerpos anticitoplasma de neutrófilos
K1 Comorbilidad
K1 Femenino
K1 Estudios de seguimiento
K1 Estudios longitudinales
K1 Masculino
K1 Mediana edad
K1 Recurrencia
K1 Estudios retrospectivos
K1 Prevalencia
K1 España
K1 Factores de tiempo
AB 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.
PB Lippincott, Williams & Wilkins
SN 0025-7974
YR 2017
FD 2017-02
LK http://hdl.handle.net/10668/2627
UL http://hdl.handle.net/10668/2627
LA en
NO Solans-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.
DS RISalud
RD Jul 1, 2025