RESUMO
BACKGROUND: Patients with focal segmental glomerulosclerosis (FSGS) are considered to have a poor prognosis and spontaneous remissions are seldom reported. However, FSGS is not a single disease entity. Our aim was to describe the clinical course in initially untreated patients with recently diagnosed idiopathic FSGS. METHODS: This was a retrospective study of patients with a diagnosis of FSGS by histology, who fulfilled the following criteria: proteinuria >3.5 g/day, normal renal function, duration of proteinuria or hypertension of less than one year, normal-sized kidneys, no underlying renal disease, and a negative family history. Renal biopsies were reviewed without knowledge of the clinical course. RESULTS: Twenty patients (13 male, 7 female) fulfilled the study criteria. Median age was 49.3 (range 21.8 to 73.0) years, serum creatinine 90 +/- 20 micromol/l, proteinuria 10.0 +/- 5.5 g/day and serum albumin 24 +/- 6 g/l. After a median follow-up of 9.4 (2.1-18.6) years, 13 patients (65%) were in remission of proteinuria. Renal function deterioration occurred in seven patients, and prompted treatment in four of them. The ten-year death-censored renal survival was 89%. Renal function deterioration and remission rate could be predicted by selectivity index, serum albumin at three months after renal biopsy and the percentage of glomeruli with segmental sclerosis. CONCLUSION: Focal glomerulosclerosis is not a single disease. Case definition using strict clinical criteria identifies a subgroup of patients with idiopathic FSGS who have a good prognosis. In the majority of these patients immunosuppressive therapy is not warranted.
Assuntos
Glomerulosclerose Segmentar e Focal/diagnóstico , Imunossupressores , Adulto , Idoso , Contraindicações , Feminino , Humanos , Falência Renal Crônica/etiologia , Masculino , Pessoa de Meia-Idade , Síndrome Nefrótica/etiologia , Prognóstico , Estudos RetrospectivosRESUMO
We describe three patients with acute renal failure after the onset of gross haematuria. In all patients a presumptive diagnosis of rapidly progressive glomerulonephritis was made and immunosuppressive therapy initiated. A renal biopsy was performed in two patients, which showed evidence of IgA nephropathy. Extracapillary proliferation was seen in a few glomeruli. The most notable abnormality was acute tubular necrosis with intraluminal erythrocytes and cell debris. In the third patient, who was known to have longstanding glomerular haematuria, acute tubular necrosis was considered likely after review of the urinary sediment. Despite the fact that immunosuppressive therapy was stopped, renal function rapidly returned to normal in all these patients. We feel that our patients and additional literature data demonstrate that in patients with glomerular disease a reversible acute renal failure can occur that is caused by acute tubular necrosis mediated by haematuria. Recognition of this entity will prevent unnecessary long-term immunosuppressive therapy.
Assuntos
Injúria Renal Aguda/etiologia , Glomerulonefrite/complicações , Hematúria/patologia , Necrose Tubular Aguda/complicações , Túbulos Renais/patologia , Adulto , Biópsia , Feminino , Humanos , Imunossupressores/uso terapêutico , Necrose Tubular Aguda/patologia , Pessoa de Meia-IdadeRESUMO
Primary hyperoxaluria type 1 (PH1) usually presents with recurrent urolithiasis, nephrocalcinosis and progressive renal failure at a relatively young age. This report describes a patient who, due to the late onset of end-stage renal disease, had been diagnosed with PH1 only after failure of his second kidney graft. Retrospectively, his vascular problems, skeletal abnormalities and cardiac arrhythmias fit the picture of severe systemic oxalosis. Possible therapeutic options are discussed.
Assuntos
Rejeição de Enxerto/etiologia , Hiperoxalúria Primária/complicações , Hiperoxalúria Primária/diagnóstico , Diagnóstico Diferencial , Progressão da Doença , Rejeição de Enxerto/terapia , Humanos , Hiperoxalúria Primária/terapia , Falência Renal Crônica/complicações , Falência Renal Crônica/diagnóstico , Falência Renal Crônica/terapia , Transplante de Rim , Masculino , Pessoa de Meia-Idade , Falha de TratamentoRESUMO
We describe a patient with rheumatoid arthritis (RA) who developed a nephrotic syndrome during treatment with a fully human recombinant monoclonal antibody against TNFalpha (adalimumab, Humira, Abbott). The proteinuria disappeared spontaneously after cessation of anti-TNFalpha treatment and relapsed after rechallenge, pointing to anti-TNFalpha as the culprit. Although renal biopsy disclosed a membranous glomerulopathy, the clinical picture was more compatible with minimal lesion glomerulopathy. The pathogenesis of this side effect is not clear; several mechanisms could in theory lead to these abnormalities.