RESUMO
Xanthogranulomatous pyelonephritis (XGP) is a rare entity characterized by the formation of inflammatory renal masses rich in macrophages loaded with lipids. It is usually secondary to repeated urinary infections and urinary obstruction due to stones, which produce slow destruction of the renal parenchyma, requiring nephrectomy. It may sometimes be associated to secondary amyloidosis that leads to the appearance of a nephrotic syndrome. We have conducted a search in the Medline database between the years 1967 and 2003 and we only found 6 cases in adults and 3 cases in pediatric patients with amyloidosis secondary to xanthogranulomatous pyelonephritis. During this same period, there are more than 570 citations that include more than 1,000 patients with isolated XGP, so that we estimate that amyloidosis that complicates a XGP should be less than 1% of all the XGP cases. We present a case of XGP in a 51 year old female patient associated to amyloidosis that initiated with nephrotic syndrome, analyzing the clinical characteristics of the 9 previous cases. We compared their clinical characteristics with those of 51 patients with xanthogranulomatous pyelonephritis without amyloidosis of a large classical series in order to characterize this clinical picture better.
Assuntos
Pielonefrite Xantogranulomatosa/diagnóstico , Amiloidose/etiologia , Feminino , Humanos , Pessoa de Meia-Idade , Pielonefrite Xantogranulomatosa/complicaçõesRESUMO
La pielonefritis xantogranulomatosa (PXG) es una rara entidad caracterizada por la formación de masas renales inflamatorias ricas en macrófagos cargados con lípidos, habitualmente secundaria a infecciones urinarias de repetición y a obstrucción urinaria por cálculos, que produce destrucción lenta del parénquima renal precisando nefrectomía. Ocasionalmente puede asociarse a amiloidosis secundaria que induce la aparición de un síndrome nefrótico. Hemos realizado una búsqueda en la base de datos Medline entre los años 1967 y 2003 y sólo hemos encontrado 6 casos en adultos y 3 en pacientes pediátricos que presentarán una amiloidosis secundaria a una PXG. En este mismo período de tiempo hay más de 570 citas que incluyen a más de 1.000 pacientes con PXG aislada, por lo que estimamos que la amiloidosis que complica a una PXG debe ser menor de un 1% de todos los casos de PXG. Presentamos un caso de PXG en una paciente de 51 años asociado a amiloidosis que comenzó con síndrome nefrótico, analizamos las características clínicas de los 9 casos previos y las comparamos con las de 51 pacientes con PXG sin amiloidosis de una serie clásica numerosa con objeto de caracterizar mejor este cuadro clínico
Xanthogranulomatous pyelonephritis (XGP) is a rare entity characterized by the formation of inflammatory renal masses rich in macrophages loaded with lipids. It is usually secondary to repeated urinary infections and urinary obstruction due to stones, which produce slow destruction of the renal parenchyma, requiring nephrectomy. It may sometimes be associated to secondary amyloidosis that leads to the appearance of a nephrotic syndrome. We have conducted a search in the Medline database between the years 1967 and 2003 and we only found 6 cases in adults and 3 cases in pediatric patients with amyloidosis secondary to xanthogranulomatous pyelonephritis. During this same period, there are more than 570 citations that include more than 1,000 patients with isolated XGP, so that we estimate that amyloidosis that complicates a XGP should be less than 1% of all the XGP cases. We present a case of XGP in a 51 year old female patient associated to amyloidosis that initiated with nephrotic syndrome, analyzing the clinical characteristics of the 9 previous cases. We compared their clinical characteristics with those of 51 patients with xanthogranulomatous pyelonephritis without amyloidosis of a large classical series in order to characterize this clinical picture better
