Síndrome pulmón-riñón: serie de casos del Hospital Universitario San Ignacio

Abstract

IntroductionPulmonary- renal syndrome (PRS) is defined as the presence of alveolar hemorrhage and rapidly progressive glomerulonephritis. It was initially described as glomerular basement membrane syndrome, although other more common causes and mechanisms are involved such as, anti-neutrophil cytoplasmic antibody-associated vasculitis (ANCA), systemic lupus erythematosus, antiphospholipid syndrome, cryoglobulinemia, and thrombotic microangiopathy. ObjectiveTo describe the experience in the treatment of patients with PRS in our center and analyze the demographic, clinical variables, and outcomes. MethodsThe databases of the Nephrology Unit and the electronic medical records of the hospital were reviewed, and a total of 14 patients with PRS between January 2009 and August 2011 were identified. ResultsA total of 14 cases managed in the intensive care unit of the hospital where analyze, of which 57% were woman and the mean age was 44 years. The most frequent diagnosis was positive ANCA vasculitis in 8 patients, lupus in 4, one case of cryoglobulinemia, and other with thrombotic microangiopathy. Most of them were treated with corticosteroids and cyclophosphamide, with 10 patients also receiving plasmapheresis. The overall mortality was 66%. ConclusionsThe results of 14 patients with PRS in our center are presented. The most common cause of this was positive ANCA vasculitis. It is an aggressive disease and its treatment included the use of steroids, cyclophosphamide and plasmapheresis.