Clinical characteristics and outcome of Spanish patients with ANCA-associated vasculitides Impact of the vasculitis type, ANCA specificity, and treatment on mortality and morbidity

Solans-Laqué, R. ; Fraile, G. ; Rodriguez-Carballeira, M. ; Caminal, L. ; Castillo, M. J. ; Martínez-Valle, F. ; Sáez, L. (Universidad de Zaragoza) ; Rios, J. J. ; Solanich, X. ; Oristrell, J. ; Pasquau, F. ; Fonseca, E. ; Zamora, M. ; Callejas, J. L. ; Frutos, B. ; Abdilla, M. ; Fanlo, P. ; García-Sánchez, I. ; López-Dupla, M. ; Sopeña, B. ; Pérez-Iglesias, A. ; Bosch, J. A.

Resumen: 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 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.

Idioma: Inglés

DOI: 10.1097/MD.0000000000006083

Año: 2017

Publicado en: Medicine 96, 8 (2017), e6083
ISSN: 0025-7974

Factor impacto JCR: 2.028 (2017)

Categ. JCR: MEDICINE, GENERAL & INTERNAL rank: 56 / 154 = 0.364 (2017) - Q2 - T2

Factor impacto SCIMAGO: 0.168 - Medicine (miscellaneous) (Q3)

Tipo y forma: Article (Published version)

Área (Departamento): Area Medicina (Dpto. Medicina, Psiqu. y Derm.)