RESUMO
OBJECTIVES: Anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (GN) is considered "pauci-immune" with absent or mild glomerular tuft staining for immunoglobulin (Ig) and/or complement. However, it is not unusual to see some immune deposits (ID) within glomeruli on immunofluorescence (IF). We determined to evaluate the prevalence and clinical significance of immune deposits in ANCA-associated GN. METHODS: We included all patients with ANCA associated vasculitis with renal biopsies between January 2002 and May 2014: granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis and renal limited vasculitis. Patients were divided into Group A: biopsy without ID (≤2+ intensity of immunostaining) and Group B: biopsy with ID (>2+ intensity of immunostaining). Serum creatinine, estimated glomerular filtration rate (eGFR) at time of the biopsy, amount of proteinuria and hematuria, requirement of dialysis and extra renal involvement were recorded. RESULTS: Fifty-three patients (75.4% females) were included. Mean age at biopsy was 66.3 years. Typical pauci-immune GN was found in 39 patients (73.5%, group A). In 14 patients (26.4%, group B) examination revealed substantial deposition of Ig or complement in the mesangium and/or along the glomerular capillary wall. The only difference comparing both groups was significantly higher proteinuria in group B (mean 1.6/24 h (SD: 10.7) vs. 0.8/24 h (SD: 7.6), p=0.0036). CONCLUSIONS: In ANCA GN at least a quarter of patients were not "pauci-immune" (26.4%). In this subgroup, immune deposits were only associated with a significantly higher proteinuria. Further basic and clinical research is needed to elucidate the significance of immune deposition in ANCA GN.
Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/imunologia , Proteínas do Sistema Complemento/análise , Glomerulonefrite/imunologia , Imunoglobulina G/análise , Glomérulos Renais/imunologia , Idoso , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/epidemiologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/fisiopatologia , Argentina/epidemiologia , Biomarcadores/sangue , Biópsia , Creatinina/sangue , Feminino , Imunofluorescência , Taxa de Filtração Glomerular , Glomerulonefrite/diagnóstico , Glomerulonefrite/epidemiologia , Glomerulonefrite/fisiopatologia , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/epidemiologia , Granulomatose com Poliangiite/imunologia , Hematúria/diagnóstico , Hematúria/epidemiologia , Hematúria/imunologia , Humanos , Glomérulos Renais/patologia , Glomérulos Renais/fisiopatologia , Masculino , Poliangiite Microscópica/diagnóstico , Poliangiite Microscópica/epidemiologia , Poliangiite Microscópica/imunologia , Prevalência , Proteinúria/diagnóstico , Proteinúria/epidemiologia , Proteinúria/imunologia , Estudos RetrospectivosRESUMO
Praziquantel was given every eight weeks for two years to children aged under six years of age, living in a Schistosoma haematobium endemic area. Infection with S. haematobium and haematuria were examined in urine and antibody profiles (IgA, IgE, IgM, IgG1, IgG2, IgG3, and IgG4) against S. haematobium adult worm and egg antigens were determined from sera collected before each treatment. Chemotherapy reduced infection prevalence and mean intensity from 51.8% and 110 eggs per 10 ml urine, respectively, before starting re-treatment programme to very low levels thereafter. Praziquantel is not accumulated after periodic administration in children. Immunoglobulin levels change during the course of treatment with a shift towards 'protective' mechanisms. The significant changes noted in some individuals were the drop in 'blocking' IgG2 and IgG4 whereas the 'protecting' IgA and IgG1 levels increased. The antibody profiles in the rest of the children remained generally unchanged throughout the study and no haematuria was observed after the second treatment. The removal of worms before production of large number of eggs, prevented the children from developing morbidity.
Assuntos
Anti-Helmínticos/uso terapêutico , Doenças Endêmicas , Praziquantel/uso terapêutico , Esquistossomose Urinária/tratamento farmacológico , Animais , Anticorpos Anti-Helmínticos/isolamento & purificação , Antígenos de Helmintos/isolamento & purificação , Criança , Seguimentos , Hematúria/imunologia , Humanos , Recidiva , Retratamento , Schistosoma haematobium/imunologia , Esquistossomose Urinária/epidemiologia , Esquistossomose Urinária/imunologia , Fatores de Tempo , Zimbábue/epidemiologiaRESUMO
Praziquantel was given every eight weeks for two years to children aged under six years of age, living in a Schistosoma haematobium endemic area. Infection with S. haematobium and haematuria were examined in urine and antibody profiles (IgA, IgE, IgM, IgG1, IgG2, IgG3, and IgG4) against S. haematobium adult worm and egg antigens were determined from sera collected before each treatment. Chemotherapy reduced infection prevalence and mean intensity from 51.8 percent and 110 eggs per 10 ml urine, respectively, before starting re-treatment programme to very low levels thereafter. Praziquantel is not accumulated after periodic administration in children. Immunoglobulin levels change during the course of treatment with a shift towards 'protective' mechanisms. The significant changes noted in some individuals were the drop in 'blocking' IgG2 and IgG4 whereas the 'protecting' IgA and IgG1 levels increased. The antibody profiles in the rest of the children remained generally unchanged throughout the study and no haematuria was observed after the second treatment. The removal of worms before production of large number of eggs, prevented the children from developing morbidity
Assuntos
Humanos , Animais , Criança , Anti-Helmínticos/uso terapêutico , Praziquantel/uso terapêutico , Esquistossomose Urinária/tratamento farmacológico , Anticorpos Anti-Helmínticos/isolamento & purificação , Antígenos de Helmintos/isolamento & purificação , Doenças Endêmicas , Seguimentos , Hematúria/imunologia , Recidiva , Retratamento , Schistosoma haematobium/imunologia , Esquistossomose Urinária/epidemiologia , Esquistossomose Urinária/imunologia , Fatores de Tempo , Zimbábue/epidemiologiaRESUMO
Characteristic ultrastructural alterations of the glomerular basement membrane (GBM) have been reported in hereditary nephritis and in children without a family history of renal disease. The clinical features, renal biopsy findings, and subsequent course were studied retrospectively in 48 children with such GBM changes to compare findings in those with and without a family history of nephritis and to determine the significance of the GBM changes in patients with nonfamilial disease. All 48 patients had hematuria. For 30, there was hematuria in at least one other member of the family (familial hematuria group); for 18, there was no familial incidence. There were no differences between the two groups with regard to clinical and pathologic findings. At the latest follow-up six boys with familial hematuria and three boys with nonfamilial hematuria had reduced renal function, and nine boys with familial hematuria and four boys and one girl with nonfamilial hematuria had neurosensory deafness. Our study results show that children with these GBM changes, with or without a family history of hematuria, tend to have a progressive course, with frequent occurrence of neurosensory deafness, and that the prognosis is more severe in boys. These observations suggest that such GBM changes in patients with nonfamilial hematuria may represent new mutations for hereditary nephritis.
Assuntos
Glomérulos Renais/patologia , Nefrite Hereditária/patologia , Membrana Basal/patologia , Criança , Pré-Escolar , Complemento C3/análise , Feminino , Seguimentos , Hematúria/imunologia , Hematúria/patologia , Humanos , Imunoglobulinas/análise , Lactente , Glomérulos Renais/imunologia , Masculino , Nefrite Hereditária/imunologia , Nefrite Hereditária/urina , Estudos RetrospectivosAssuntos
Glomerulonefrite/epidemiologia , Doença Aguda , Adolescente , Adulto , Fatores Etários , beta-Globulinas/análise , Criança , Pré-Escolar , Feminino , Glomerulonefrite/diagnóstico , Glomerulonefrite/genética , Hematúria/diagnóstico , Hematúria/epidemiologia , Hematúria/imunologia , Hospitalização , Humanos , Imunodifusão , Índia , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Proteinúria/diagnóstico , Proteinúria/epidemiologia , Proteinúria/imunologia , Grupos Raciais , Trinidad e TobagoRESUMO
Incidental urines from 2019 members of the family households of 369 patients hospitalized wih acute glomerulonephritis, 1605 residents of a village, and 1299 school-children were tested with Hema-Combistix. (In this report, the family of a patient is termed a nephritis family.) Hematuria was more frequent in nephritis family members under 10 years old than in similiar control subjects. Proteinuria was more frequent in nephritis family members aged 10 to 30 and 40 to 60 years than in similiar village residents. The control school-children (tested at school) had proteinuria more frequently than did nephritis family children (tested at home). Urine abnormalities were found in 219 of the nephritis family members. Subtracting similiar urine abnormalities found in the control groups from those of the nephritis family members leaves 98 of these abnormalities presumably attributed to family asociation with currently hospitalized cases of acute nephritis. Serum á-1-C gloulin was decreased in 16 of the nephritis family members with urine abnormalities and in only one of 207 subjects from the control groups. Decreased serum á-1-C globulin was associated with mild stigmata of acute glomerulonephritis in the nephritis family members (AU)