Comportamiento clínico y factores asociados a mortalidad temprana en una cohorte de pacientes chilenos con vasculitis asociadas a anticuerpos anti citoplasma de neutrófilos (VAA) / Variables associated with mortality in 103 patients with anti-neutrophil cytoplasmic antibodies associated vasculitis

Background: Cumulative survival in patients with anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis (VAA) is 88 and 78% at 1 and 5 years, respectively. Despite this, mortality continues to be 2.7 times higher than the general population. Differences in the clinical profile of VAA in different ethnicities have been observed. Aim: To identify factors at the time of diagnosis, associated with mortality at one year of follow-up and to describe the clinical characteristics of these patients. Material and Methods: We identified in local databases and reviewed clinical records of patients with VAA with at least one year of follow up in a clinical hospital. Demographic and laboratory parameters and clinical activity scores were analyzed. Results: Of 103 patients with VAA identified, 65 met the inclusion criteria and were analyzed. Their age ranged from 45 to 63 years and 56% were women. Thirty-five patients (54%) were diagnosed as granulomatosis with Polyangiitis (GPA) and 30 patients (46%) with Microscopic Polyangiitis (MPA). The frequency of renal disease was 53% and pulmonary involvement occurred in 72%. At one year of follow-up 11 patients died resulting in a mortality of 17%. Seven patients died within three months after diagnosis. MPO ANCA were more common than PR3 ANCA. In the multivariate analysis, the presence of ophthalmological involvement, lung kidney syndrome and a Five Factor Score (FFS) of 1 or more were independent factors associated with mortality at one year. Conclusions: In these patients, pulmonary manifestations predominate. Lung kidney syndrome, ophthalmological involvement and a FFS score ≥ 1 were associated with mortality.

Arteritis de celulas gigantes: clásicos y novedades que vale la pena recordar / Giant cells arteritis: classics and innovations for remember

La Arteritis de Células Gigantes (ACG) es una vasculitis frecuente que ocurre en personas mayores y que afecta principalmente vasos craneanos. Generalmente se acompaña de síntomas sistémicos, claudicación mandibular y alteraciones visuales. La Polimialgia Reumática (PMR) se caracteriza por dolor y rigidez de cintura escapular y pelviana que presenta síntomas constitucionales y reacciones sistémicas. En los últimos años, ha aparecido evidencia que relaciona ambas entidades como componentes de una misma enfermedad. En este artículo se revisan aspectos nuevos en diagnóstico, terapia y etiopatogenia de la ACG y sus relaciones con PMR.

The Giant cell arteritis (GCA) is a common vasculitic syndrome occurring in older persons and it preferentially affects cranial arteries. Generally accompanied by constitutional symptoms and typical findings like jaw claudication and vision disorder.Polymyalgia Rheumatica (PMR) is caracterized by pain and stiffness involving shoulder and pelvic girdless with constitutional symptoms and findings of a systemic reaction. In recents years, evidence linking both conditions as components of asingle disease process has been accumulated. In the present article, we review new aspects of the diagnosis, therapy and pathogenesis of the GCA, and their relationship.