RESUMEN
Abstract One of the most common causes of rapidly progressive glomerulonephritis (RPGN) is pauci-immune crescentic glomerulonephritis (CrGN). In the majority of cases, this condition has a positive serologic marker, the anti-neutrophil cytoplasmic antibodies (ANCAs), but in approximately 10% there are no circulating ANCAs, and this subgroup has been known as the ANCA-negative pauci-immune CrGN. RPGN can be associated with systemic diseases, but there are only few case reports describing the association with mixed connective tissue disease (MCTD). The authors report a case of ANCA-negative CrGN associated with a MCTD.
Resumo Uma das causas mais comuns da glomerulonefrite rapidamente progressiva (GNRP) é a glomerulonefrite crescêntica (GNC) pauci-imune. Na maioria dos casos, a patologia apresenta um marcador sorológico positivo, o anticorpo anticitoplasma de neutrófilos (ANCA), mas em cerca de 10% dos pacientes não há ANCAs circulantes, perfazendo um subgrupo da patologia conhecido como GNC pauci-imune ANCA-negativa. A GNRP pode estar associada a doenças sistêmicas, mas são poucos os relatos de caso que descrevem sua associação com doença mista do tecido conjuntivo (DMTC). O presente artigo relata um caso de GNC ANCA-negativa associada a DMTC.
Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Anticuerpos Anticitoplasma de Neutrófilos , Glomerulonefritis/complicaciones , Enfermedad Mixta del Tejido Conjuntivo/complicaciones , Glomerulonefritis/inmunología , Glomerulonefritis/patología , Riñón/patología , Glomérulos Renales/patología , Enfermedad Mixta del Tejido Conjuntivo/inmunologíaRESUMEN
One of the most common causes of rapidly progressive glomerulonephritis (RPGN) is pauci-immune crescentic glomerulonephritis (CrGN). In the majority of cases, this condition has a positive serologic marker, the anti-neutrophil cytoplasmic antibodies (ANCAs), but in approximately 10% there are no circulating ANCAs, and this subgroup has been known as the ANCA-negative pauci-immune CrGN. RPGN can be associated with systemic diseases, but there are only few case reports describing the association with mixed connective tissue disease (MCTD). The authors report a case of ANCA-negative CrGN associated with a MCTD.
Asunto(s)
Anticuerpos Anticitoplasma de Neutrófilos , Glomerulonefritis/complicaciones , Enfermedad Mixta del Tejido Conjuntivo/complicaciones , Glomerulonefritis/inmunología , Glomerulonefritis/patología , Humanos , Riñón/patología , Glomérulos Renales/patología , Masculino , Persona de Mediana Edad , Enfermedad Mixta del Tejido Conjuntivo/inmunologíaRESUMEN
ABSTRACT Atheroembolic renal disease (AERD) is a kidney manifestation of atherosclerosis as a systemic disease. AERD is defined as a renal impairment secondary to embolization of cholesterol crystals with consequent occlusion of renal vascularization. The current case report describes one patient with multiple risk factors but without any inciting event history who presents a very atypical clinical course of a severe and massive atheroembolic disease that developed spontaneously and silently.
RESUMO A doença renal ateroembólica (DRAE) é uma manifestação renal da aterosclerose enquanto patologia sistêmica. A DRAE é definida como uma disfunção renal secundária à embolização de cristais de colesterol seguida da oclusão da vascularização renal. O presente relato descreve o caso de um paciente com vários fatores de risco, porém sem um evento precipitante, que se apresentou com um curso clínico bastante atípico de doença ateroembólica grave de evolução espontânea e silenciosa.
Asunto(s)
Humanos , Masculino , Anciano , Insuficiencia Renal/diagnóstico por imagen , Aterosclerosis/complicaciones , Dislipidemias/complicaciones , Hipertensión/complicaciones , Biopsia , Inhibidores de Agregación Plaquetaria/uso terapéutico , Hipertrigliceridemia , Aspirina/uso terapéutico , Inhibidores de Hidroximetilglutaril-CoA Reductasas/uso terapéutico , Creatinina/sangre , Diabetes Mellitus Tipo 2/complicaciones , Nefropatías Diabéticas/etiología , Insuficiencia Renal/etiología , Clopidogrel/uso terapéutico , Hipercolesterolemia , Riñón/patología , Microscopía , Antiinflamatorios/uso terapéuticoRESUMEN
Atheroembolic renal disease (AERD) is a kidney manifestation of atherosclerosis as a systemic disease. AERD is defined as a renal impairment secondary to embolization of cholesterol crystals with consequent occlusion of renal vascularization. The current case report describes one patient with multiple risk factors but without any inciting event history who presents a very atypical clinical course of a severe and massive atheroembolic disease that developed spontaneously and silently.