Síndrome pulmón-riñón: serie de 12 casos / Pulmonary renal syndrome: series of 12 cases

El síndrome pulmón-riñón es una entidad infrecuente, que comprende un gran espectro de patologías, como las vasculitis asociadas a ANCA y la enfermedad por anticuerpos antimembrana basal glomerular entre otras. Se describen en esta serie 12 casos donde las entidades más prevalentes fueron las antes mencionadas, observándose además un caso de lupus y uno de granulomatosis con poliangeítis, que se encuentran dentro de las causas menos frecuentes. La forma de presentación clínica inicial fue simultánea renal y pulmonar en 5/12 pacientes y renal en 7/12 de los mismos. El diagnóstico temprano de dichas patologías basándose en criterios clínicos, radiológicos, de laboratorio e histológicos, permite instaurar terapéuticas tempranas como la inmunosupresión y plasmaféresis, pudiendo prevenir complicaciones tales como las infecciones y la insuficiencia renal crónica terminal, siendo las primeras la principal causa de muerte (AU)

Pulmonary-renal syndrome is an infrequent condition. It includes a wide variety of conditions such as ANCA (antineutro-phil cytoplasmic autoantibody) associated with systemic vasculitis and anti-GBM (anti-glomerular basement membrane) disease among others. In this series we describe twelve cases, in which the most prevalent diseases were the above mentioned as well as one case of lupus and one of granulomatosis with polyangiitis (these being less frequent causes). The clinical presentation was both renal and pulmonary simultaneously in five of twelve patients and renal in seven of twelve patients. Early diagnosis of this condition on the basis of clinical, radiological, histological and analytic criteria allows early treatments such as immunosuppression and plasma exchange, thus avoiding complications such as infections (the main cause of death) and terminal chronic renal failure (AU)

Granulomatosis de Wegener. A propósito de un caso / Wegener's granulomatosis: a case report andr review of the literature

Las enfermedades vasculares se encuentran divididas para su mejor comprensión en patologías de vasos grandes medianos o pequeños calibres; en este apartado nos dedicamos a revisar un caso específico de enfermedad granulomatosa de Wegener que corresponde directamente a una enfermedad de pequeños vasos, que comprometió los ojos, los oídos, los pulmones, los riñones y la piel; logrando reunir la parte clínica, patología, las imágenes diagnósticas, laboratorios y el manejo farmacológico. Se debe tener en cuenta que los sistemas de mayor compromiso son el respiratorio y el renal no dejando a un lado el otorrino la piel, el gastrointestinal y sistema nervioso entre otros. Siendo de importancia la pesquisa de los ANCA (anticuerpos anticitoplasma de neutrófilos) siendo el más específico el pr3 (proteinasa3) y la biopsia anatomopatológica, la cual confirma el diagnostico

Vascular diseases are divided for better understanding in pathologies of medium large vessels or small calibers; in this section, we review a specific case of Wegener's granulomatous disease that corresponds directly to a disease of small vessels, which affected the eyes, ears, lungs, kidneys and skin; managing to gather the clinical part, pathology, diagnostic images, laboratories, pharmacological management. It must be taken into account that the systems of greatest commitment are the respiratory and the renal, not leaving aside the otorrino skin, gastrointestinal and nervous system among others. The ANCA (antineutrophil cytoplasmic antibodies) is of importance, with the most specific being pr3 (proteinase3) and the anatomopathological biopsy, which confirms the diagnosis

Compromiso renal en vasculitis asociadas a anticuerpos anticitoplasma de neutrófilos. Recomendaciones de consenso de las Sociedades Chilenas de Nefrología y Reumatología / Renal involvement in antineutrophil cytoplasmic antibodies (ANCA) associated vasculitides. Recommendations of the Chilean Societies of Nephrology and Rheumatology

Renal involvement is a frequent complication in antineutrophil cytoplasmic antibodies (ANCA)associated vasculitides, adding morbidity and mortality, such as chronic kidney disease and the need for renal replacement therapy. With the aim of reaching a consensus on relevant issues regarding the diagnosis, treatment and follow-up of patients with these diseases, the Chilean Societies of Nephrology and Rheumatology formed a working group that, based on a critical review of the available literature and their experience, raised and answered consensually a set of questions relevant to the subject. This document includes aspects related to the clinical diagnosis, the histological characteristics, the therapeutic alternatives to induce and maintain the remission of the disease, relapse surveillance strategies and complementary therapies.

Patogênese e tratamento da glomerulonefrite - uma atualização / Pathogenesis and treatment of glomerulonephritis-an update

Resumo A presente revisão traz os conceitos mais atuais acerca dos fatores de risco genéticos, eventos etiológicos, respostas nefritogênicas e tratamento dos principais tipos de glomerulonefrite (GN) imunomediada. Tais patologias incluem GN pós-infecciosa, nefropatia por IgA, doença por anticorpo antimembrana basal glomerular (anti-MBG), vasculite associada a ANCA (VAA) e nefrite lúpica. Apesar da(s) etiologia(s) da maioria dos casos de GN permanecer indefinida, acredita-se que seu início se deva, em grande parte, a insultos ambientais, particularmente na forma de processos infecciosos que deflagram respostas de hospedeiro em indivíduos geneticamente suscetíveis, levando assim a quadros de GN. A concepção mecanicista em torno dessas patologias evoluiu a partir da visão mais antiga de que a maioria seria consequência do aprisionamento glomerular de complexos imunes pré-formados para a percepção atual de que as mesmas, em sua maioria, são doenças autoimunes por natureza mediadas por anticorpos e linfócitos T reativos a auto-antígenos. O tratamento da GN não tem acompanhado os progressos na compreensão de sua patogênese. Os papéis recentemente atribuídos a mediadores mais antigos como complemento e proteínas reguladoras do complemento lançam luz sobre novos alvos terapêuticos.

Abstract This review updates current concepts of the genetic risk factors, etiologic events, nephtitogenic responses and treatment of the major immunologically mediated types of glomerulonephritis (GN). These include post-infectious GN, IgA nephropathy, anti-glomerular basement membrane (GBM) antibody disease, ANCA-associated vasculitis (AAV) and lupus nephritis. Although the etiology(s) of most GNs remain undefined, many are now believed to be initiated by environmental insults, particularly infectious processes, that trigger host responses in genetically susceptible individuals which lead to GN. Mechanistic concepts of these diseases have evolved from earlier views that most were consequent to glomerular trapping of preformed immune complexes to the current view that most of these diseases are auto-immune in nature mediated by both antibodies and T cells reactive with self-antigens. Therapy of GN has lagged behind advances in understanding pathogenesis. Newly appreciated roles for older mediators like complement and complement regulatory proteins offer new therapeutic targets.