Clinical characteristics and outcome of Spanish patients with ANCA-associated vasculitides: Impact of the vasculitis type, ANCA specificity, and treatment on mortality and morbidity. Issue 8 (February 2017)

• Record Type: Journal Article
• Authors: Solans-Laqué, Roser; Fraile, Guadalupe; Rodriguez-Carballeira, Monica; Caminal, Luis; Castillo, Maria J.; Martínez-Valle, Ferran; Sáez, Luis; Rios, Juan J.; Solanich, Xavier; Oristrell, Joaquim; Pasquau, Francisco; Fonseca, Eva; Zamora, Monica; Callejas, Jose L.; Frutos, Begoña; Abdilla, Monica; Fanlo, Patricia; García-Sánchez, Isabel; López-Dupla, Miguel; Sopeña, Bernardo; Pérez-Iglesias, Almudena; Bosch, Josep A.
• Other Names: Hajji. Raouf section editor.
• Is Part Of: Medicine. Volume 96:Issue 8(2017)
• Publication Date: 2017-02
• Subjects: ANCA-associated vasculitides -- eosinophilic granulomatosis with polyangiitis -- granulomatosis with polyangiitis -- infections -- microscopic polyangiitis -- mortality predictors -- outcome -- Spanish people; Medicine -- Periodicals; Medicine -- Periodicals; Médecine -- Périodiques; Geneeskunde; Medicine; Periodicals; Periodicals; 610.5
• DOI: 10.1097/MD.0000000000006083
• Languages: English
• ISSNs: 0025-7974

Abstract

Abstract : 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.