RESUMO
Background: Tolvaptan (TV) is the first vasopressin-receptor antagonist approved for the treatment of autosomal dominant polycystic kidney disease (ADPKD). No publications report TV experience in real clinical practice during the first year of treatment. Methods: A prospective study of an initial cohort of 220 rapidly progressing patients treated with TV for 12 months. The tolerability of TV, the evolution of the estimated glomerular filtration rate (eGFR), analytical parameters, and blood pressure were analyzed. Results: A total of 163 patients (78.2%) received TV for 1 year. The main causes of treatment withdrawal were the aquaretic effects (11%), eGFR deterioration (5%), and hepatic toxicity (2.3%). eGFR decreased significantly after 1 month of treatment without further changes. The decrease in eGFR in the first month was higher in patients with an initially higher eGFR. The eGFR drop during the first year of treatment with TV was lower than that reported by patients in the 2 years prior to TV treatment (-1.7 ± 7.6 vs. -4.4 ± 4.8 mL/min, p = 0.003). Serum sodium and uric acid concentrations increased, and morning urinary osmolality decreased in the first month, with no further changes. Blood pressure decreased significantly without changes in antihypertensive medication. Conclusion: TV treatment is well tolerated by most patients. Liver toxicity is very rare and self-limited. TV reduces eGFR in the first month without showing further changes during the first year of treatment. Patients with a higher starting eGFR will suffer a greater initial drop, with a longer recovery. We suggest using the eGFR observed after a month of treatment as the reference for future comparisons and calculating the rate of eGFR decline in patients undergoing TV treatment.
RESUMO
El síndrome hemofagocítico es una condición clínica e histológica grave, secundaria a diferentes procesos. La glomerulonefritis colapsante es una podocitopatía proliferativa, generalmente de pronóstico desfavorable para la función renal. Se presenta un caso en el que las dos condiciones aparecieron asociadas, lo cual es una forma infrecuente de presentación del linfoma hepatoesplénico de células T. Se discute, asimismo, el papel de los marcadores de desdiferenciación podocitaria en esta glomerulopatía, y se revisan la fisiopatología y el tratamiento.
The hemophagocytic syndrome is a serious clinical-histological entity secondary to different diseases. Collapsing glomerulonephritis is a proliferative podocytopathy that usually has an unfavorable renal prognosis. We present a case in which both entities were associated, which is an infrequent form of hepatosplenic T-cell lymphoma. In addition, we review the role of the markers of podocyte dedifferentiation in this glomerulopathy and its pathophysiology and treatment.
Assuntos
Linfo-Histiocitose Hemofagocítica , Glomerulonefrite , Antígenos de Diferenciação , Insuficiência Renal , Linfoma , Transtornos LinfoproliferativosRESUMO
The hemophagocytic syndrome is a serious clinical-histological entity secondary to different diseases. Collapsing glomerulonephritis is a proliferative podocytopathy that usually has an unfavorable renal prognosis. We present a case in which both entities were associated, which is an infrequent form of hepatosplenic T-cell lymphoma. In addition, we review the role of the markers of podocyte dedifferentiation in this glomerulopathy and its pathophysiology and treatment.
El síndrome hemofagocítico es una condición clínica e histológica grave, secundaria a diferentes procesos. La glomerulonefritis colapsante es una podocitopatía proliferativa, generalmente de pronóstico desfavorable para la función renal. Se presenta un caso en el que las dos condiciones aparecieron asociadas, lo cual es una forma infrecuente de presentación del linfoma hepatoesplénico de células T. Se discute, asimismo, el papel de los marcadores de desdiferenciación podocitaria en esta glomerulopatía, y se revisan la fisiopatología y el tratamiento.
Assuntos
Desdiferenciação Celular , Glomerulonefrite/patologia , Podócitos/patologia , Glomerulonefrite/complicações , Humanos , Neoplasias Hepáticas/complicações , Linfo-Histiocitose Hemofagocítica/etiologia , Linfoma de Células T/complicações , Masculino , Pessoa de Meia-Idade , Neoplasias Primárias Múltiplas/complicações , Neoplasias Esplênicas/complicaçõesRESUMO
No disponible
Assuntos
Humanos , Nefrite Lúpica/tratamento farmacológico , Lúpus Eritematoso Sistêmico/complicações , Insuficiência Renal/prevenção & controle , Padrões de Prática Médica , Nefrite Lúpica/complicações , Corticosteroides/administração & dosagem , Quimioterapia de Manutenção , Falha de TratamentoAssuntos
Imunossupressores/uso terapêutico , Nefrite Lúpica/tratamento farmacológico , Guias de Prática Clínica como Assunto , Injúria Renal Aguda/tratamento farmacológico , Injúria Renal Aguda/etiologia , Algoritmos , Relação Dose-Resposta a Droga , Quimioterapia Combinada , Humanos , Imunossupressores/administração & dosagem , Nefrite Lúpica/classificação , Nefrite Lúpica/complicações , Síndrome Nefrótica/tratamento farmacológico , Síndrome Nefrótica/etiologia , Pulsoterapia , Fatores de Risco , Terapia de Salvação , Índice de Gravidade de Doença , Sociedades Médicas , EspanhaRESUMO
No disponible
