Clinical characteristics and outcome of Spanish patients with ANCA-associated vasculitides: Impact of the vasculitis type, ANCA specificity, and treatment on mortality and morbidity
التفاصيل البيبلوغرافية
العنوان:
Clinical characteristics and outcome of Spanish patients with ANCA-associated vasculitides: Impact of the vasculitis type, ANCA specificity, and treatment on mortality and morbidity
Spanish Registry of systemic vasculitis (REVAS) from the Autoimmune Diseases Study Group (GEAS) of the Spanish Society of Internal Medicine (SEMI), [Solans-Laqué,R, Martínez-Valle,F, Bosch,JA] Hospital Valle Hebrón, Barcelona. [Fraile,G] Hospital Ramón y Cajal, Madrid. [Rodriguez-Carballeira,M] Mutua Terrassa, Barcelona. [Caminal,L] Hospital Central de Asturias. [Castillo,MJ] Hospital Virgen del Rocío, Sevilla. [Sáez,L] Hospital Miguel Servet, Zaragoza. [Rios,JJ] Hospital La Paz, Madrid. [Solanich,X] Hospital Bellvitge. [Oristrell,J] Hospital Parc Tauli Sabadell, Barcelona. [Pasquau.F] Hospital Marina Baixa, Villajoyosa, Alicante. [Fonseca,E] Hospital Cabueñes, Asturias. [Zamora,M] Hospital Virgen de las Nieves. [Callejas,JL] Hospital Clínico San Cecilio, Granada. [Frutos,B] Hospital Fuenlabrada, Madrid. [Abdilla,O] Hospital La Ribera, Alzira, Valencia. [Fanlo,P] Clínica de Navarra. [García-Sánchez,I] Hospital Infanta Leonor, Madrid. [López-Dupla,M] Hospital Joan XXIII, Tarragona. [Sopeña,B] Centro Hospitalário Vigo. [Pérez-Iglesias,A] Hospital Ourense, Galicia, Spain. [Bosch,JA] Spanish Registry of systemic vasculitis (REVAS) from the Autoimmune Diseases Study Group (GEAS) of the Spanish Society of Internal Medicine (SEMI)., Institut Català de la Salut, [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., Vall d'Hebron Barcelona Hospital Campus
المصدر:
Repositorio Institucional de la Consejería de Sanidad de la Comunidad de Madrid Consejería de Sanidad de la Comunidad de Madrid MEDICINE r-FISABIO. Repositorio Institucional de Producción Científica instname r-FISABIO: Repositorio Institucional de Producción Científica Fundación para el Fomento de la Investigación Sanitaria y Biomédica de la Comunitat Valenciana (FISABIO) Zaguán. Repositorio Digital de la Universidad de Zaragoza Medicine Scientia
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.
