1. Clinical characteristics and outcome of Spanish patients with ANCA-associated vasculitides: Impact of the vasculitis type, ANCA specificity, and treatment on mortality and morbidity
- Author
-
Solans-Laqué R, Fraile G, Rodriguez-Carballeira M, Caminal L, Castillo MJ, Martínez-Valle F, Sáez L, Rios JJ, Solanich X, Oristrell J, Pasquau F, Fonseca E, Zamora M, Callejas JL, Frutos B, Abdilla M, Fanlo P, García-Sánchez I, López-Dupla M, Sopeña B, Pérez-Iglesias A, Bosch JA, Spanish Registry of systemic vasculitis (REVAS) from the Autoimmune Diseases Stu, 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., and Vall d'Hebron Barcelona Hospital Campus
- Subjects
Diseases::Pathological Conditions, Signs and Symptoms::Pathologic Processes::Disease Attributes::Recurrence [Medical Subject Headings] ,Male ,Other subheadings::Other subheadings::/epidemiology [Other subheadings] ,calidad, acceso y evaluación de la atención sanitaria::calidad de la atención sanitaria::factores epidemiológicos::comorbilidad [ATENCIÓN DE SALUD] ,Phenomena and Processes::Physical Phenomena::Time::Time Factors [Medical Subject Headings] ,España ,Comorbidity ,Estudios longitudinales ,ANCA-associated vasculitides ,Gastroenterology ,Anticuerpos anticitoplasma de neutrófilos ,Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::Humans [Medical Subject Headings] ,0302 clinical medicine ,Longitudinal Studies ,Young adult ,Anciano de 80 o más años ,Masculino ,Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Vital Statistics::Morbidity::Prevalence [Medical Subject Headings] ,Adolescente ,Aged, 80 and over ,General Medicine ,eosinophilic granulomatosis with polyangiitis ,Geographic Locations::Europe::Spain [GEOGRAPHICALS] ,Cohort ,Microscopic polyangiitis ,Chemicals and Drugs::Amino Acids, Peptides, and Proteins::Proteins::Blood Proteins::Immunoproteins::Immunoglobulins::Antibodies::Autoantibodies::Antibodies, Antineutrophil Cytoplasmic [Medical Subject Headings] ,Vasculitis asociada a anticuerpos citoplasmáticos antineutrófilos ,medicine.medical_specialty ,mortality predictors ,Anciano ,Recurrencia ,Check Tags::Male [Medical Subject Headings] ,03 medical and health sciences ,Estudios retrospectivos ,Other subheadings::Other subheadings::Other subheadings::/mortality [Other subheadings] ,Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Epidemiologic Study Characteristics as Topic::Epidemiologic Studies::Cohort Studies::Longitudinal Studies [Medical Subject Headings] ,Humans ,Persons::Persons::Age Groups::Adult [Medical Subject Headings] ,Aged ,Retrospective Studies ,Mediana edad ,Otros calificadores::Otros calificadores::Otros calificadores::/mortalidad [Otros calificadores] ,granulomatosis with polyangiitis ,Eosinophilic granulomatosis with polyangiitis ,Persons::Persons::Age Groups::Adult::Middle Aged [Medical Subject Headings] ,Mortality predictors ,medicine.disease ,Diseases::Immune System Diseases::Autoimmune Diseases::Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis [Medical Subject Headings] ,Check Tags::Female [Medical Subject Headings] ,Persons::Persons::Age Groups::Adult::Aged::Aged, 80 and over [Medical Subject Headings] ,Granulomatosis with polyangiitis ,Other subheadings::Other subheadings::/physiopathology [Other subheadings] ,Time Factors ,Cardiovascular Diseases::Vascular Diseases::Vasculitis::Systemic Vasculitis::Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis [DISEASES] ,Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Epidemiologic Study Characteristics as Topic::Epidemiologic Studies::Case-Control Studies::Retrospective Studies [Medical Subject Headings] ,Adulto joven ,Recurrence ,Health Care::Health Care Quality, Access, and Evaluation::Quality of Health Care::Epidemiologic Factors::Comorbidity [Medical Subject Headings] ,Prevalence ,030212 general & internal medicine ,infections ,Persons::Persons::Age Groups::Adult::Aged [Medical Subject Headings] ,microscopic polyangiitis ,Adulto ,Otros calificadores::Otros calificadores::/epidemiología [Otros calificadores] ,Femenino ,Middle Aged ,Comorbilidad ,enfermedades cardiovasculares::enfermedades vasculares::vasculitis::vasculitis sistémica::vasculitis asociada a anticuerpos anticitoplasma de neutrófilos [ENFERMEDADES] ,Treatment Outcome ,outcome ,Ubicaciones Geográficas::Europa (Continente)::España [DENOMINACIONES GEOGRÁFICAS] ,Female ,Estudios de seguimiento ,Vasculitis ,Persons::Persons::Age Groups::Adult::Young Adult [Medical Subject Headings] ,medicine.drug ,Research Article ,Adult ,Otros calificadores::Otros calificadores::/fisiopatología [Otros calificadores] ,Cyclophosphamide ,Adolescent ,Spanish people ,Observational Study ,Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ,Infections ,Persons::Persons::Age Groups::Adolescent [Medical Subject Headings] ,Antibodies, Antineutrophil Cytoplasmic ,Young Adult ,Comorbiditat ,Internal medicine ,Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Epidemiologic Study Characteristics as Topic::Epidemiologic Studies::Cohort Studies::Longitudinal Studies::Follow-Up Studies [Medical Subject Headings] ,medicine ,Health Care Quality, Access, and Evaluation::Quality of Health Care::Epidemiologic Factors::Comorbidity [HEALTH CARE] ,Analytical, Diagnostic and Therapeutic Techniques and Equipment::Diagnosis::Prognosis::Treatment Outcome [Medical Subject Headings] ,030203 arthritis & rheumatology ,Geographical Locations::Geographic Locations::Europe::Spain [Medical Subject Headings] ,business.industry ,Retrospective cohort study ,Surgery ,Outome ,Spain ,Factores de tiempo ,Vasculitis - Espanya ,Resultado del tratamiento ,business ,Prevalencia ,Follow-Up Studies - 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.
- Published
- 2017