Annals of the Rheumatic Diseases | 2019
AB0585\u2005REJA-VASCA: JAVERIAN REGISTRY OF PATIENTS WITH POSITIVE ANCA VASCULITIS AT THE UNIVERSITY HOSPITAL SAN IGNACIO 2005–2017
Abstract
Background: Vasculitis associated with cytoplasmic antibodies (VAAC) are heterogeneous entities with low prevalence and high morbidity and mortality. These include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (PAM), and eosinophilic granulomatosis with polyangiitis (GEPA). European retrospective records report an annual incidence of 10-20 cases per million, with peak presentation between 65-74 years, predominantly in males. It has diverse clinical manifestations in multiple systems. In Colombia, there are few clinical records that describe the clinical characteristics of these patients. Objectives: To describe the clinical characteristics of patients with VAAC in the University Hospital San Ignacio between 2005-2017 Methods: Descriptive cross-sectional study. Patient records were reviewed between January 2005 and December 2017. The records were obtained using search systems in patients who were requested Antineutrophil cytoplasmic antibodies - ANCAS (Searching softeare: DISEARCH, LABCORE), obtaining 2072 results. 106 patients met the criteria for classification of the American College of Rheumatology for VAAC. Results: 106 patients diagnosed with ANCA positive vasculitis. Average age 55 years (± 14.7). 48.4% were women. In-hospital debut in 68.8%. The average number of days of stay was 16.6 days (± 12.22), 56 patients (52.8%) had granulomatosis with polyangiitis GPA, 45 patients (42.4%) with microscopic polyangiitis (MAP) and 5 patients with eosinophilic granulomatosis with polyangiitis EGP (4.8%). 37 patients (35%) presented with alveolar hemorrhage, 22 patients (20.7%) presented renal compromise with rapidly progressive glomerulonephritis. Central nervous system compromise was documented in 25 patients (24%). Regarding treatment, 97 patients (91.5%) received treatment with intravenous or oral corticosteroid, 66 patients (62.2%) received treatment with cyclophosphamide. Plasma exchange therapy was performed in 15 patients (14.1%). 44 patients (41.5%) required renal replacement therapy. The in-hospital mortality of this group of patients was 20.4%, with sepsis being the most frequent cause of death (55%). Conclusion: In this study, the most frequent positive ANCA vasculitis was granulomatosis with polyangiitis. Multisystemic compromise was present in all groups of patients, the most common being pulmonary and renal, mainly in patients with microscopic polyangiitis, similar to that reported by Solans-Laqué et al in Spain. Within the demographic characteristics, the average age at the time of diagnosis is around 50 years, predominantly in women related to the Mexican study by Hinojosa-Azaola et al. The most frequent cause of in-hospital mortality was sepsis. Clinical information of patients with VAAC is presented in a reference hospital in Colombia. Data are concordant with literature. Disclosure of Interests: None declared