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INTRODUCTION: Although RT has improved the survival of the population with ESRD due to all causes, renal outcomes in SLE are controversial. The objective of this study is to describe the characteristics and evolution of the patients and the kidney transplant in LN, and compare it with patients transplanted for other causes. MATERIALS AND METHODS: Retrospective, observational, analytical, single-center study in which records of patients undergoing nephrotransplantation for LN were analyzed. They were compared with a group of patients transplanted at the same center for other causes of ESRD. RESULTS: 41 patients with kidney transplant due to SLE and 89 transplanted due to other causes of ESRD were registered. Graft loss occurred in 12 (29.26%) patients with LN and 34 (38.2%) patients in the comparison group (p = .428). Only one case (4.8%) presented reactivation of the LN in the graft, without graft loss. Median graft survival was 73.1 months in the LN group and 66.3 months in the comparison group (p = .221). A total of 8 (19.5%) patients with LN and 11 (12.4%) without LN died (p = .42), with infections being the main cause in both groups. There were no statistically significant differences between groups in graft and patient survival. In a sub-analysis of 28 patients with LN with aPL study, 4 thrombotic events were observed, in 3 different patients, in the aPL-positive group. There were no statistically significant differences in terms of causes of graft loss and graft survival (positive aFL 75.7 months vs negative aFL 72.7 months, p= .96). There were also no differences in mortality between the groups (p = .61). CONCLUSION: Patients transplanted for LN did not differ from the control population in terms of graft and patient survival. Infections were the main cause of death, so prophylaxis and vaccination continue to be a fundamental pillar in the prevention of infections in immunocompromised patients.
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Supervivencia de Injerto , Fallo Renal Crónico , Trasplante de Riñón , Nefritis Lúpica , Humanos , Estudios Retrospectivos , Femenino , Nefritis Lúpica/cirugía , Nefritis Lúpica/mortalidad , Nefritis Lúpica/complicaciones , Adulto , Masculino , Argentina/epidemiología , Fallo Renal Crónico/cirugía , Fallo Renal Crónico/mortalidad , Persona de Mediana Edad , Pronóstico , Adulto Joven , Rechazo de Injerto , Resultado del TratamientoAsunto(s)
Factores Inmunológicos , Inmunosupresores , Glomérulos Renales , Lupus Eritematoso Sistémico , Nefritis Lúpica , Manejo de Atención al Paciente , Progresión de la Enfermedad , Humanos , Factores Inmunológicos/inmunología , Factores Inmunológicos/uso terapéutico , Inmunosupresores/clasificación , Inmunosupresores/inmunología , Inmunosupresores/uso terapéutico , Glomérulos Renales/patología , Glomérulos Renales/fisiopatología , Trasplante de Riñón/métodos , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/epidemiología , Lupus Eritematoso Sistémico/terapia , Nefritis Lúpica/inmunología , Nefritis Lúpica/mortalidad , Nefritis Lúpica/fisiopatología , Nefritis Lúpica/terapia , Monitoreo Fisiológico/métodos , Gravedad del Paciente , Manejo de Atención al Paciente/métodos , Manejo de Atención al Paciente/normas , Pronóstico , Inducción de Remisión/métodos , Factores de Riesgo , Resultado del TratamientoRESUMEN
INTRODUCTION: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease associated with high mortality rates. This study aimed to describe the main causes of death in a case series of SLE patients attended in a single center in Colombia. METHODS: We conducted a retrospective review and analysis of records of SLE patients who died between January 2011 and June 2017. We extracted the main causes of death and described variables associated with this outcome as well as variables associated with the disease and its treatment. RESULTS: From a total of 1776 patients with SLE, we identified 49 fatal cases (89.8% women, n = 44). The average age at death was 40.6 years (SD 17.4), and patients had a median of 4.5 years (IQR 2-8) of disease duration. The main findings included lymphopenia in 44 patients (89.9%), biopsy-confirmed lupus nephritis (LN)-types IV and VI-in 38 (77.6%), catastrophic antiphospholipid syndrome (CAPS) in 8 (16.3%), and persistent hypocomplementemia (C3 and C4) in 8 (16.3%). The median SLE disease activity index (SLEDAI-2K) score at the time of death was 19 (IQR 11-39). The main cause of death was SLE activity and lupus-induced damage in 22 (44.9%) patients. CONCLUSION: The main causes of death included SLE activity refractory to immunosuppressive treatment, and nosocomial bacterial infections. The patients who died had persistently high SLEDAI scores, types IV and VI LN, associated antiphospholipid syndrome, and persistent hypocomplementemia, requiring severe immunosuppression and prolonged hospitalization.
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Lupus Eritematoso Sistémico/mortalidad , Adolescente , Adulto , Síndrome Antifosfolípido/complicaciones , Síndrome Antifosfolípido/mortalidad , Colombia/epidemiología , Femenino , Humanos , Inmunosupresores/uso terapéutico , Infecciones/complicaciones , Infecciones/mortalidad , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/tratamiento farmacológico , Nefritis Lúpica/complicaciones , Nefritis Lúpica/mortalidad , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Trombocitopenia/complicaciones , Trombocitopenia/mortalidad , Adulto JovenRESUMEN
Kidney transplant for patients with lupus nephritis (LN) has satisfactory outcomes in studies with short-term or mid-term follow up. Nevertheless, information about long-term outcomes is scarce. We performed a retrospective matched-pair cohort study in 74 LN recipients compared with 148 non-LN controls matched by age, sex, immunosuppressive treatment, human leukocyte antigen (HLA) matches, and transplant period in order to evaluate long-term outcomes of kidney transplant in LN recipients. Matched pairs were predominantly females (83%), median age at transplant surgery of 32 years (interquartile range 23-38 years), and 66% received a graft from a living related donor. Among LN recipients, 5-, 10-, 15-, and 20-year graft survival was 81%, 79%, 57% and 51%, respectively, and it was similar to that observed in controls (89%, 78%, 64%, and 56%, respectively). Graft loss (27% vs. 21%, p = 0.24) and overall survival ( p = 0.15) were not different between LN recipients and controls. Also, there was no difference in episodes of immunological rejection, thrombosis, or infection. Only six LN recipients had biopsy-proven lupus recurrence and three of them had graft loss. In a cohort with a long follow up of kidney transplant recipients, LN recipients had similar long-term graft survival and overall outcomes compared with non-lupus recipients when predictors are matched between groups.
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Supervivencia de Injerto , Inmunosupresores/uso terapéutico , Trasplante de Riñón , Nefritis Lúpica/mortalidad , Nefritis Lúpica/terapia , Adulto , Femenino , Humanos , Estimación de Kaplan-Meier , Masculino , México , Estudios Retrospectivos , Centros de Atención Terciaria , Factores de Tiempo , Adulto JovenRESUMEN
BACKGROUND: Systemic lupus erythematosus (SLE) is a severe autoimmune disease that involves multiple organ systems. Lupus nephritis (LN) is a complication of SLE and is associated with poor survival and high morbidity. Many genomic studies have been performed worldwide, and several histocompatibility leukocyte antigen (HLA) loci are linked to lupus susceptibility. OBJECTIVE: The present study evaluated the association of HLA alleles in a lupus patient population, LN group and control group. The second objective evaluated whether HLA allele match or mismatch influenced kidney graft survival in a kidney transplanted lupus population. METHODS: This study was a retrospective study of 2 major groups: general lupus patients (GSLE - nâ¯=â¯108) and a control group (GControl - nâ¯=â¯216). Both groups were also divided into subgroups. RESULTS: The control group was divided into two subgroups: a healthy control group (HeCTRL) and transplant control group (TxCTRL). The GSLE group was composed of transplanted lupus patients (TxSLE) and non-transplanted lupus patients (nTxSLE). Comparison of the demographics between groups did not reveal differences between ethnicity and gender. A difference in the prevalence of three alleles, B*08, DRB1*08 and DRB1*15, was observed. These alleles were more prevalent in the lupus subgroups compared to the control groups. Five-year survival was not different between patients carrying the allele DRB1*15 in either group (overall pâ¯=â¯0.075; TxSLE pâ¯=â¯0.419; TxCTRLâ¯=â¯0.309). The presence of the match with this allele in the receptor was evaluated and did not demonstrate any difference in graft survival in both groups (pâ¯=â¯0.146) or when analyzed separately in each group (TxCTRL pâ¯=â¯0.739; TxSLEâ¯=â¯0.297). CONCLUSION: This study demonstrated that the presence of HLA-DRB1*15 was a strong factor that predisposed patients to the development of SLE and LN, but did not influence kidney graft survival.
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Genotipo , Rechazo de Injerto/genética , Antígenos HLA/genética , Cadenas HLA-DRB1/genética , Trasplante de Riñón , Lupus Eritematoso Sistémico/genética , Nefritis Lúpica/genética , Alelos , Etnicidad , Femenino , Frecuencia de los Genes , Estudios de Asociación Genética , Predisposición Genética a la Enfermedad , Rechazo de Injerto/mortalidad , Supervivencia de Injerto , Humanos , Lupus Eritematoso Sistémico/mortalidad , Lupus Eritematoso Sistémico/terapia , Nefritis Lúpica/mortalidad , Nefritis Lúpica/terapia , Masculino , Polimorfismo Genético , Estudios Retrospectivos , Factores Sexuales , Análisis de SupervivenciaRESUMEN
Background Lupus nephritis (LN) is one of the most frequent complications of SLE and occurs in up to 50% of cases depending on the studied population. Of these, approximately 20% progress to end-stage renal disease (ESRD), with the treatment of choice being a kidney transplant. Objective The objective of this study was to describe the clinical outcome of patients transplanted due to LN, compared with patients transplanted for other causes, in a Latin American population from the Fundación Valle del Lili in Cali, Colombia. Methods Observational, retrospective case study with controls matched by age, sex and type of donor in a single center between 1996 and 2014. Results Sixty-five kidney transplants were performed in patients with LN and ESRD. The survival of patients with LN was 98% at 1, 10 and 15 years ( p = .99). For controls by age and sex, survival was also 98% at 15 years post-transplant, and for controls by donor, the survival rate was 100% at 5 years and 98% at 15 years. Graft survival in patients with LN to 1, 5 and 15 years was 92%, 83% and 71%, respectively; for controls by age and sex, it was 90%, 84% and 64%, respectively, and for the controls by donor, it was 89%, 86% and 79%, respectively ( p = .7718). There were no statistically significant differences found in the cumulative incidence of acute graft rejection in the first year, but it was found that acute rejection is a factor that relates to the loss of function of the renal graft ( p = .032). Of the patients transplanted for LN, two (3.1%) experienced a recurrence of the disease. One patient died after a diagnosis of recurrence of LN due to an infection. Conclusions Kidney transplantation is a good option for patients with ESRD due to LN. In this Hispanic population, the survival of patients, graft survival, and cumulative incidence of graft rejection are not different from those of other transplanted patients. In addition, recurrence of LN was rare, showing the benefits of renal transplantation in LN patients with ESRD.
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Fallo Renal Crónico/cirugía , Trasplante de Riñón , Nefritis Lúpica/cirugía , Enfermedad Aguda , Adulto , Colombia , Femenino , Rechazo de Injerto/epidemiología , Supervivencia de Injerto , Humanos , Incidencia , Estimación de Kaplan-Meier , Fallo Renal Crónico/diagnóstico , Fallo Renal Crónico/mortalidad , Trasplante de Riñón/efectos adversos , Trasplante de Riñón/mortalidad , Nefritis Lúpica/diagnóstico , Nefritis Lúpica/mortalidad , Masculino , Persona de Mediana Edad , Seguridad del Paciente , Complicaciones Posoperatorias/epidemiología , Prevalencia , Recurrencia , Estudios Retrospectivos , Factores de Riesgo , Factores de Tiempo , Resultado del TratamientoRESUMEN
The clinical expression of systemic lupus erythematosus (SLE) is influenced by genetic and environmental factors and therefore varies between ethnicities. Information on the epidemiology of SLE in Brazil is scarce and practically limited to studies conducted in socioeconomically developed regions (South and Southeast). The objective of this study was to describe the clinical and immunological aspects and outcome of a cohort of patients with SLE treated at a university hospital in northeastern Brazil and compare patterns related to age at onset: childhood (cSLE), adult (aSLE), and late (lSLE). A random sample of 414 records (women: 93.5%) were reviewed. The mean age at SLE onset and the mean disease duration were 28.9 ± 10.9 years and 10.2 ± 6.6 years, respectively. Most patients had aSLE (n = 338; 81.6%), followed by cSLE (n = 60; 14.5%) and lSLE (n = 16; 3.9%). The female/male ratio was 6.5:1 in cSLE and 16.8:1 in aSLE; in lSLE, all patients were female (p = 0.05). During follow-up, the cSLE group presented higher rates of nephritis (70% vs. 52.9% vs. 12.5%; p = 0.0001) and leuko/lymphopenia (61.7% vs. 43.8% vs. 56.2%; p = 0.02). No significant differences were found for anti-dsDNA, anti-Sm, and antiphospholipid antibodies. Treatment with immunosuppressants was significantly more common, and higher doses of prednisone were used, in cSLE. The prevalence of cardiovascular diseases were more frequent in lSLE (p = 0.03). No significant differences were found between the three groups with regard to mean damage accrual (SDI), remission, and mortality. Although cSLE presented higher rates of nephritis and leuko/lymphopenia, more frequent use of immunosuppressants and higher prednisone doses than aSLE and lSLE, the three groups did not differ significantly with regard to damage accrual, remission, and mortality.
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Lupus Eritematoso Sistémico/inmunología , Nefritis Lúpica/inmunología , Adolescente , Adulto , Edad de Inicio , Anticuerpos Antinucleares/sangre , Anticuerpos Antifosfolípidos/sangre , Biomarcadores/sangre , Brasil/epidemiología , Niño , Preescolar , Comorbilidad , Progresión de la Enfermedad , Femenino , Glucocorticoides/uso terapéutico , Hospitales Universitarios , Humanos , Inmunosupresores/uso terapéutico , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Lupus Eritematoso Sistémico/mortalidad , Nefritis Lúpica/diagnóstico , Nefritis Lúpica/tratamiento farmacológico , Nefritis Lúpica/mortalidad , Masculino , Persona de Mediana Edad , Prevalencia , Inducción de Remisión , Estudios Retrospectivos , Factores de Riesgo , Factores de Tiempo , Resultado del Tratamiento , Adulto JovenRESUMEN
OBJECTIVE: To evaluate response rates in an adult lupus nephritis (LN) cohort in Mexico City, Mexico. METHODS: We analyzed 165 patients with biopsy-proven LN histological International Society of Nephrology/Renal Pathology Society classes III, IV, or V, distributed by treatment drug in 3 groups: mycophenolate mofetil (MMF; dosage > 2 g/day per 6 mos, n = 63), intravenous cyclophosphamide (IVC; 0.7 g/m(2) body surface area monthly per 6 pulses, n = 66), or azathioprine (AZA; dosage > 1.5 mg/kg/day per 6 mos, n = 36). Median followup was 31 ± 18 months. The primary endpoint was the proportion of patients achieving complete renal response (CR). Secondary endpoints included the proportion of patients achieving renal response (complete or partial), renal flare-free survival, doubling of serum creatinine, and progression to endstage renal disease (ESRD). RESULTS: MMF induction was superior to IVC (HR 2.00, 95% CI 1.23-3.25, p = 0.005) and AZA (HR 2.12, 95% CI 1.23-3.66, p = 0.007) in the primary endpoint. Censored CR rates at 6, 12, 24, and 36 months were 32.6%, 56.1%, 76.6%, and 94.1% for MMF; 24.2%, 34.4%, 57.9%, and 62.1% for IVC; and 8.4%, 39.8%, 49.7%, and 49.7% for AZA. MMF was also superior in renal response to treatment and renal flare-free survival outcomes. There were no differences between groups in doubling of serum creatinine or progression to ESRD. The induction treatment with MMF (HR 2.04, 95% CI 1.25-3.33, p = 0.005) and absence of vascular lesions on renal biopsy (HR 2.05, 95% CI 1.25-3.37, p = 0.004) were associated with CR, whereas proteinuria at the time of presentation was negatively associated with CR (HR 0.91, 95% CI 0.84-0.98, p = 0.013). CONCLUSION: MMF induction therapy is superior to IVC and AZA in patients with LN of Mexican-mestizo race.
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Azatioprina/uso terapéutico , Ciclofosfamida/uso terapéutico , Inmunosupresores/uso terapéutico , Nefritis Lúpica/tratamiento farmacológico , Nefritis Lúpica/patología , Ácido Micofenólico/análogos & derivados , Administración Oral , Corticoesteroides/uso terapéutico , Adulto , Análisis de Varianza , Biopsia con Aguja , Estudios de Cohortes , Supervivencia sin Enfermedad , Femenino , Estudios de Seguimiento , Hospitales de Enseñanza , Humanos , Inmunohistoquímica , Inmunosupresores/efectos adversos , Infusiones Intravenosas , Estimación de Kaplan-Meier , Pruebas de Función Renal , Nefritis Lúpica/mortalidad , Masculino , México , Análisis Multivariante , Ácido Micofenólico/uso terapéutico , Modelos de Riesgos Proporcionales , Inducción de Remisión , Estudios Retrospectivos , Medición de Riesgo , Índice de Severidad de la Enfermedad , Tasa de Supervivencia , Resultado del Tratamiento , Adulto JovenRESUMEN
OBJECTIVE: To define if antibodies to ribosomal P proteins disclose a better lupus nephritis long-term survival. METHODS: Sixty consecutive SLE patients with biopsy-proven nephritis (2004 ISN/RPS) were evaluated for renal survival parameters. Inclusion criteria were at least one serum sample at: renal flares, biopsy, and last follow-up until 2008. Anti-P was detected by ELISA/immunoblot and anti-dsDNA by indirect immunofluorescence/ELISA. RESULTS: Eleven patients (18%) with anti-P+ (without anti-dsDNA) during renal flare were compared to 49 (82%) persistently negative for anti-P throughout the study. At the final follow-up post-biopsy (6.3±2.5 vs. 6.8±2.4 years, p=0.36), the comparison of anti-P+/anti-dsDNA- with anti-P- group revealed a trend to lower mean creatinine levels (0.9±0.3 vs. 2.3±2.1 mg/dl, p=0.07), lower frequency of dialysis (0% vs. 35%, p=0.025), and higher frequency of normal renal function (91% vs. 53%, p=0.037). The overall renal survival was significantly higher in anti-P+/anti-dsDNA- compared to anti-P- (11.0±4.5 vs. 9.2±4.5 years, p=0.033), anti-dsDNA+/anti-P- (vs. 8.7±4.7 years, p=0.017), and anti-P-/anti-dsDNA- (vs. 9.8±4.3 years, p=0.09) groups. CONCLUSION: Our data supports the notion that anti-P antibody in the absence of anti-dsDNA during nephritis flares is a valuable marker to predict a better long-term renal outcome in lupus patients.
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Anticuerpos Antinucleares/sangre , Nefritis Lúpica/inmunología , Nefritis Lúpica/mortalidad , Proteínas Ribosómicas/inmunología , Adulto , Anticuerpos Antinucleares/inmunología , Biomarcadores/sangre , Supervivencia sin Enfermedad , Femenino , Humanos , Riñón/inmunología , Riñón/metabolismo , Riñón/fisiopatología , Pruebas de Función Renal , Nefritis Lúpica/sangre , Nefritis Lúpica/fisiopatología , Nefritis Lúpica/terapia , Masculino , Valor Predictivo de las Pruebas , Tasa de SupervivenciaRESUMEN
OBJECTIVE: Damage accrual in SLE has been previously shown to be an independent predictor of mortality. We sought to discern which SLICC Damage Index (SDI) domains are the most important predictors of survival in SLE. METHODS: SLE patients (ACR criteria), age > or =16 years, disease duration < or =5 years at enrolment, of African-American, Hispanic or Caucasian ethnicity were studied. Disease activity was assessed using the SLAM-Revised (SLAM-R) at diagnosis. Damage was ascertained using the SDI at the last visit. The SDI domains associated with time to death (and interaction terms) were examined by univariable and multivariable Cox proportional hazards regression analyses; those significant in the multivariable analyses were added to the final two models (with and without poverty) that included other variables known to be associated with shorter survival. RESULTS: A total of 635 SLE patients were studied of whom 97 (15.3%) have died over a mean (s.d.) total disease duration of 5.7 (3.7) years. Patients were predominantly women [570 (89.8%)]; their mean (s.d.) age was 36.5 (12.6) years; 126 (19.8%) had developed renal damage, 62 (9.3%) cardiovascular, 48 (7.8%) pulmonary and 34 (5.4%) peripheral vascular damage. When excluding poverty from the multivariable model, the renal domain of the SDI was independently associated with a shorter time to death (hazard ratio = 1.65; 95% CI 1.03, 2.66). CONCLUSIONS: The renal domain of the damage index is associated with a shorter time to death when poverty, a strong predictor of this outcome, is removed from the model. Preventing renal damage in lupus patients has long-term prognostic implications.
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Nefritis Lúpica/mortalidad , Adolescente , Adulto , Negro o Afroamericano/estadística & datos numéricos , Anciano , Métodos Epidemiológicos , Femenino , Hispánicos o Latinos/estadística & datos numéricos , Humanos , Lupus Eritematoso Sistémico/etnología , Lupus Eritematoso Sistémico/mortalidad , Nefritis Lúpica/etnología , Masculino , Persona de Mediana Edad , Pobreza/estadística & datos numéricos , Pronóstico , Puerto Rico/epidemiología , Factores Sexuales , Texas/epidemiología , Adulto JovenRESUMEN
Systemic lupus erythematosus (SLE) is a clinical syndrome of varying severity. Although the survival and prognosis of SLE have steadily improved, there is a group of patients who present an acute fatal outcome despite aggressive therapy. We designed this study to evaluate the factors associated with mortality in patients with acute severe SLE. During 2004-06, 41 Mexican SLE patients that could not be managed in the out-patient clinic and with acute severe major organ system involvement [nephritis, severe thrombocytopenia (platelet count below 20 000 per microL) acute neuropsychiatric pulmonary, gastrointestinal or cardiac disease and generalized vasculitis] were studied. During the first admission, disease activity (SLE Disease Activity Index (SLEDAI), SLE Activity Measured), damage [SLE International Collaborating Clinics (SLICC)], and therapy were assessed. Survival using Kaplan-Meier curves, odd ratios with 95% confidence interval and logistic regression analysis were used to determine risk factors for mortality. Ninety percent were female with a mean age of 29 +/- 19 years and mean disease duration of 21 +/- 9 months. The principal causes of first admission were renal (27%), SNC (22%) and cardiopulmonary (15%). After a mean follow-up of 9.7 +/- 6 months, 16 (39%) patients died. Deceased patients had significantly higher SLEDAI (P = 0.004), and SLICC (P = 0.03) scores. The manifestations associated with mortality were renal disease activity (odds ratio, OR 4.6, confidence interval, CI 95% 1.0-20.6), infections (OR 3.2 CI 95% 2.0-5.3) and thrombocytopenia (OR 4.0, CI 95% 1.0-15.9). The survival at 9.7 months was 72, 62 and 50% in patients with an SLEDAI score of 3-10, 11-20 and > or =21, respectively. The SLEDAI score, the presence of damage and infection were associated with death in patients with acute severe SLE.
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Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/mortalidad , Índice de Severidad de la Enfermedad , Enfermedad Aguda , Adolescente , Adulto , Estudios de Cohortes , Hospitalización , Humanos , Infecciones/complicaciones , Infecciones/mortalidad , Estimación de Kaplan-Meier , Nefritis Lúpica/mortalidad , Masculino , México/epidemiología , Oportunidad Relativa , Trombocitopenia/complicaciones , Trombocitopenia/mortalidadRESUMEN
The objective of this study was to analyse the survival rate and cause of death in children with systemic lupus erythematosus (SLE) during the past 30 years in Chile. A retrospective analysis was performed between 1969 and 2000 on patients attending pediatric rheumatology centres in Santiago, Chile. Survival and causes of death in 31 children followed from 1969 to 1980 fulfilling the 1982 American College of Rheumatology criteria for SLE and treated with oral steroids were compared with 50 other patients who were treated with oral steroids and an aggressive treatment of IV bolus of cyclophosphamide (38 patients) and azathioprine (12 patients). Global survival at five and 10 years follow-up for the patients studied from 1969 to 1980 was 68 and 40%, respectively. During the second study period these values were significantly improved and global survival reached 95% at five years and 90% at 10 years follow-up (P < 0.05). Survival at 10 years follow-up for patients with lupus nephropathy increased from 28% (study period 1964-1980) to 86% (study period 1984-2000). Twelve children died (38%) during the 1964-1980 study period. The causes of death were six due to kidney failure, three due to infectious conditions and another three of unknown causes. During the 1980-2000 study period mortality reached 6% (three cases), two cases died of a lupus flare-up and one case due to infection. In the last three decades, we have seen an important increase in the survival of children with SLE, especially in those patients with renal involvement. Management with immunosuppressive drugs, such as IV cyclophosphamide or azathioprine has changed the prognosis in these children. These results demonstrate that our children with SLE increased their life expectancy but are now faced with new types of morbidity because of the sequelae related to the disease itself.
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Lupus Eritematoso Sistémico/mortalidad , Adolescente , Niño , Chile/epidemiología , Femenino , Estudios de Seguimiento , Humanos , Inmunosupresores/uso terapéutico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Nefritis Lúpica/tratamiento farmacológico , Nefritis Lúpica/mortalidad , Masculino , Pronóstico , Estudios Retrospectivos , Análisis de SupervivenciaRESUMEN
BACKGROUND: The long-term outcome of the pure form of WHO type V lupus membranous glomerulonephritis is apparently more benign than that of other forms of lupus glomerulonephritis. However 12% of such patients progress to terminal renal failure. The presence of proteinuria may be an indication of cytotoxic agents. AIM: To study the clinical long-term outcome of WHO type V lupus membranous glomerulonephritis. MATERIAL AND METHODS: A retrospective analysis of all kidney biopsies of a University Pathology Department, with the diagnosis of WHO type V lupus membranous glomerulonephritis. Review of medical records of patients with the disease and one clinical assessment of all living patients. RESULTS: Between 1973 and 2000, 703 kidney biopsies were done to patients with systemic lupus erythematosus. Of these, 40 were membranous glomerulonephritis and in 33 patients (28 women, age range 6-71 years), data on the evolution and survival was obtained. Nineteen had type Va and the rest type Vb nephritis. Two presented with renal failure and 11 with proteinuria over 3.5 g/24 h. The median follow-up since the renal biopsy was 63 months (range 1-316). At the end of follow-up, four had a creatinine clearance of less then 15 ml/h and four a clearance between 15 and 29 ml/h (one of these received a renal allograft). Eleven (33%) patients had died, mostly due to infections. Life expectancy at five years with a creatinine clearance over 15 ml/h was 75%. Bad prognostic factors were an elevated creatinine clearance over 15 ml/h was 75%. Bad prognostic factors were an elevated creatinine and high blood pressure at the moment of the biopsy. CONCLUSIONS: The clinical outcome of these patients was bad. Twelve percent reached a stage of terminal renal failure. This is in contrast with the 3% progression to a similar stage of proliferative glomerulonephritis treated with i.v. cyclophosphamide. New therapies for this condition must be sought.
Asunto(s)
Glomerulonefritis Membranosa , Nefritis Lúpica , Adolescente , Adulto , Anciano , Biopsia , Niño , Chile/epidemiología , Femenino , Estudios de Seguimiento , Glomerulonefritis Membranosa/tratamiento farmacológico , Glomerulonefritis Membranosa/mortalidad , Glomerulonefritis Membranosa/patología , Humanos , Nefritis Lúpica/tratamiento farmacológico , Nefritis Lúpica/mortalidad , Nefritis Lúpica/patología , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Análisis de SupervivenciaRESUMEN
OBJECTIVE: To analyze clinical, laboratory and treatment features associated with death in a childhood-onset SLE population. PATIENTS AND METHODS: Patients with childhood-onset SLE followed at the State University of Campinas, Brazil, between 1980 and 2002 were included. Data on clinical and laboratory features of the disease were collected regularly. Logistic regression was used for analyzing association between clinical and laboratory features and death. Kaplan-Meyer tests were used to estimate the survival curves. RESULTS: Of 61 patients identified, six were lost to follow-up during the first year of disease. The mean follow-up period of the remaining 55 patients was 3.25 years (SD=1.2). Mean SLICC/ACR-DI score was 4.9 (SD=3.4). Death occurred in 12 (21.8%) of 55 patients. Direct causes of death were: infection in six (50%), stroke in four (33.3%), and renal insufficiency in two (16.7%). Five patients (41.7%) died during the first 5 years of disease due to infection. Male gender (p=0.004; OR=9.1; 95% CI=7.6-21.0), infection (p=0.001; OR=4.2; 95% CI=1.6-15.2) and nephritis (p=0.02; OR=2.3; 95% CI=1.3-5.2) were independent factors associated with death in the multivariate analysis. The global survival rate adjusted for duration of disease was 93.9% in the first year of disease, 88.9% in the second year, 80.8% in the fifth year and 48.1% in 20 years of follow-up. When comparing survival curves, male gender, the presence of infection during the course of the disease and the presence of nephritis during follow-up had a worse survival. CONCLUSION: Male gender, the presence of infection and nephritis were independent risk factors for death in our Brazilian cohort. Damage did not independently influence survival in this study.
Asunto(s)
Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/mortalidad , Adolescente , Factores de Edad , Brasil/epidemiología , Niño , Estudios de Cohortes , Femenino , Humanos , Infecciones/etiología , Infecciones/mortalidad , Lupus Eritematoso Sistémico/terapia , Nefritis Lúpica/etiología , Nefritis Lúpica/mortalidad , Masculino , Pronóstico , Factores Sexuales , Análisis de SupervivenciaRESUMEN
Background: The long-term outcome of the pure form of WHO type V lupus membranous glomerulonephritis is apparently more benign than that of other forms of lupus glomerulonephritis. However 12percent of such patients progress to terminal renal failure. The presence of proteinuria may be an indication of cytotoxic agents. Aim: To study the clinical long-term outcome of WHO type V lupus membranous glomerulonephritis. Material and methods: A retrospective analysis of all kidney biopsies of a University Pathology Department, with the diagnosis of WHO type V lupus membranous glomerulonephritis. Review of medical records of patients with the disease and one clinical assessment of all living patients. Results: Between 1973 and 2000, 703 kidney biopsies were done to patients with systemic lupus erythematosus. Of these, 40 were membranous glomerulonephritis and in 33 patients (28 women, age range 6-71 years), data on the evolution and survival was obtained. Nineteen had type Va and the rest type Vb nephritis. Two presented with renal failure and 11 with proteinuria over 3.5 g/24h. The median follow-up since the renal biopsy was 63 months (range 1-316). At the end of follow-up, four had a creatinine clearance of less then 15 ml/h and four a clearance between 15 and 29 ml/h (one of these received a renal allograft). Eleven (33percent) patients had died, mostly due to infections. Life expectancy at five years with a creatinine clearance over 15 ml/h was 75percent. Bad prognostic factors were an elevated creatinine clearance over 15 ml/h was 75percent. Bad prognostic factors were an elevated creatinine and high blood pressure at the moment of the biopsy. Conclusions: The clinical outcome of these patients was bad. Twelve percent reached a stage of terminal renal failure. This is in contrast with the 3percent progression to a similar stage of proliferative glomerulonephritis treated with i.v. cyclophosphamide. New therapies for this condition must be sought.
Asunto(s)
Adolescente , Adulto , Masculino , Humanos , Femenino , Niño , Persona de Mediana Edad , Glomerulonefritis Membranosa/mortalidad , Glomerulonefritis Membranosa/patología , Glomerulonefritis Membranosa/tratamiento farmacológico , Nefritis Lúpica/mortalidad , Nefritis Lúpica/patología , Nefritis Lúpica/tratamiento farmacológico , Biopsia , Chile/epidemiología , Estudios de SeguimientoRESUMEN
Environmental, genetic, and ethnic factors seem to be involved in the expression of systemic lupus erythematosus (SLE). It has been reported that SLE in the Spanish-heritage population is more common and severe than in the white population in the United States. Data on the population with SLE in South America are scarce, however. The survival of 218 Chilean patients with SLE was studied. General features of the disease were similar to those in most reports. Survival at 10 years was 79%. Prognostic factors (P < .01) were the clinical markers of renal severity, including clinical stratification of renal disease, SLE disease activity index, thrombocytopenia, thromboembolism, and the use of "megadose" corticosteroids. Multivariate analysis suggested that the disease activity index had the strongest association with outcome (P = .0007); thrombocytopenia added a marginal risk (P = .04). Renal histology was analyzed in 127 patients. Risk factors for survival were clinical renal stage, elevated serum creatinine levels, and high activity index. Chronicity index was not a risk factor. Survival curves for World Health Organization types II and III were better than for type IV (P = .06). Multivariate analysis showed that creatinine levels correlated inversely with outcome (P = .0363). Disease expression of Chilean patients with SLE was similar to that in most reports. Survival was somewhat lower than in developed countries, which may be attributable to socioeconomic and racial factors.
Asunto(s)
Lupus Eritematoso Sistémico/mortalidad , Adolescente , Adulto , Anciano , Causas de Muerte , Niño , Chile/epidemiología , Femenino , Humanos , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/tratamiento farmacológico , Nefritis Lúpica/sangre , Nefritis Lúpica/mortalidad , Masculino , Persona de Mediana Edad , Pronóstico , Índice de Severidad de la Enfermedad , Tasa de SupervivenciaRESUMEN
This study reports the severity of clinical lupus nephritis (LN), defined as the sustained presence of proteinuria and/or cellular casts, in a group of 68 newly diagnosed patients with systemic lupus erythematosus (SLE) in Curaçao. Fifty-four patients (78%) developed clinical signs of LN, of whom 31 (54%) had clinical LN at the time of SLE diagnosis. The probability of developing clinical LN reached 80% in the first 3 years after SLE diagnosis and hardly increased later. No clinical or serological differences existed at the time of SLE diagnosis or at onset of LN between patients with early-or late-onset LN. Survival in patients without LN was 100% at 5 years, while for patients with clinical LN these rates at 1 and 5 years were 91% and 59%, respectively (P = 0.0001); male LN patients had a worse prognosis than females (P = 0.012), while time of LN onset did not influence survival. Six patients (11%) developed end-stage renal failure; all were female, five had early LN and one had late-onset LN (P = 0.17). Renal survival was 97% and 80% at 1 and 5 years, with decreased rates for patients with nephrotic-range proteinuria (P = 0.02). Hypertension was present in 13% of LN patients, but had no influence on patient or renal survival. Thus, clinical LN was a frequent complication, which carried a poor prognosis in these Afro-Caribbean lupus patients.
Asunto(s)
Nefritis Lúpica/etnología , Adulto , Población Negra , Femenino , Humanos , Riñón/fisiopatología , Nefritis Lúpica/mortalidad , Nefritis Lúpica/fisiopatología , Masculino , Persona de Mediana Edad , Factores Socioeconómicos , Tasa de Supervivencia , Indias OccidentalesRESUMEN
This study reports the severity of clinical lupus nephritis (LN), defined as the sustained presence of proteinuria and/or cellular casts, in a group of 68 newly diagnosed patients with systemic lupus erythematosus (SLE) in Curacao. Fifty-four patients (78 percent) developed clinical signs of LN, of whom 31 (54 percent) had clinical LN at the time of SLE diagnosis. The probability of developing clinical LN reached 80 percent in the first 3 years after SLE diagnosis and hardly increased later. No clinical or serological differences existed at the time of SLE diagnosis or at onset of LN between patients with early-or late-onset LN. Survival in patients without LN was 100 percent at 5 years, while for patients with clinical LN these rates at 1 and 5 years were 91 percent and 59 percent, respectively (P= 0.0001); male LN patients had a worse prognosis than females (P=0.012), while time of LN onset did not influence survival. Six patients (11 percent) developed end-stage renal failure; all were female, five had early LN and one had late-onset LN (P=0.17). Renal survival was 97 percent and 80 percent at 1 and 5 years, with decreased rates for patients with nephrotic-range proteinuria (P=0.02). Hypertension was present in 13 percent of LN patients, but had no influence on patient or renal survival. Thus, clinical LN was a frequent complication, which carried a poor prognosis in these Afro-Caribbean lupus patients (AU)
Asunto(s)
Humanos , Adulto , Masculino , Femenino , Nefritis Lúpica/etnología , Riñón/fisiopatología , Nefritis Lúpica/mortalidad , Nefritis Lúpica/fisiopatología , Tasa de Supervivencia , Indias Occidentales , Factores SocioeconómicosRESUMEN
Lupus nephritis has a high mortality and morbidity rate at the General Hospital, Port-of-Spain. We analysed 52 consecutive systemic lupus erythematosus patients with clinical nephritis, seen at the renal unit between January, 1983 and October, 1987. There were 44 females and 8 males. Evidence for nephritis was the presence of urinary casts, haematuria, proteinuria or an elevated creatinine level. In Trinidad, this is a disease of the young African female. The modal age group was 20-29 yrs. 71 percent of patients were of African descent, 15 percent Indian and 14 percent of mixed ethnicity. All the male patients were African. East Indian females presented with nephropathy late in the course of lupus. Proteinuria was the most frequent presentation (78 percent). End-stage renal failure was seen in 14 percent of patients at presentation. Such late presentation could have been avoided if patients with epilepsy, psychiatric disturbance and resolving proteinuria had been investigated for lupus. Classes II (37 percent) and V (23 percent) were the most frequent histological patterns. The 1-yr survival rate, over a 23-month follow-up period, was 66 percent. This low figure reflects defects in our renal substitution therapy rather than aggressive disease; uraemia (14), sepsis (7), extra renal activity (4) and hypoglycaemia (1) were the causes of death. During the short follow-up, we saw no vascular complications. Sixty per cent of dialysed patients died, after a mean time of 3.5 months, from uraemia or peritoneal dialysis-related sepsis. We could recommend maintenance haemodialysis (AU)