Correlación anátomo-clínica entre clases y subclases roliferativas de nefritis lúpica en biopsias renales: experiencia de un centro único / Clinicopathological correlation among proliferative classes and subclasses of lupus nephritis in renal biopsies

Background Lupus nephritis (LN) is a complication of systemic lupus erythematosus that requires renal biopsy (RB). Proliferative classes III, IV-S, IV-G have especial clinical and pathological characteristics. Aim To determine the association between pathological features in RB with serum creatinine and urine protein levels. Material and Methods We analyzed 186 RB performed in adults aged 18 to 73 years, from a renal pathology reference center. Histopathological variables such as class and subclass of proliferative LN, endocapillary and extracapillary proliferation, activity and chronicity indexes, and vascular sclerosis were correlated with serum creatinine and urine protein levels, at the time of diagnosis. Results As compared with LN III, all the morphological and laboratory values were significantly more deteriorated in LN IV, with special focus on vascular sclerosis. Serum creatinine was the only variable that significantly differentiated LN IV-S from LN IV-G. Proteinuria was non-significantly higher in LN IV-G compared to LN IV-S. However, the difference became significant when proteinuria was compared between LN IV-G and LN III. Conclusions The significant difference in serum creatinine between LN IV-S and LN IV-G supports the concept that they are different subclasses. Proteinuria is a variable that differentiates classes III from IV-G, being significantly higher in the second. Severe arteriosclerosis is a constant and significant finding that differentiates LN III from LN IV. Thus, we propose its usefulness for distinguishing LN classes, and eventually, to be considered in the chronicity index.

Pediatric lupus nephritis / Nefrite lúpica em pediatria

Abstract Involvement of the kidneys by lupus nephritis (LN) is one of the most severe clinical manifestations seen in individuals with systemic lupus erythematosus (SLE). LN is more frequent and severe in pediatric patients and has been associated with higher morbidity and mortality rates. This narrative review aimed to describe the general aspects of LN and its particularities when affecting children and adolescents, while focusing on the disease's etiopathogenesis, clinical manifestations, renal tissue alterations, and treatment options.

Resumo A nefrite lúpica (NL) é caracterizada pelo acometimento dos rins no contexto das diversas manifestações clínicas do Lupus Eritematoso Sistêmico (LES), e representa uma das manifestações clínicas mais graves da doença. A NL é mais frequente e mais grave nos pacientes pediátricos, em comparação com os adultos, e causa maiores taxas de morbidade e mortalidade. O objetivo desta revisão narrativa foi descrever os aspectos gerais da NL e suas particularidades em crianças e adolescentes, com foco em sua etiopatogênese, nas manifestações clínicas, nas alterações histopatológicas renais e na abordagem terapêutica.

Características clínicas y hallazgos histopatológicos de glomerulonefritis lúpica en pacientes con Rhupus en el Hospital Nacional Arzobispo Loayza en los años 2003 al 2009 / Clinical features and histopathological findings in Rhupus patients developing glomerulonephritis in Arzobispo Loayza Nacional Hospital between 2003 and 2009

Hasta el 10% de pacientes con Lupus Eritematoso sistémico tienen la artropatía similar a Artritis Reumatoide, denominado ôRuphusõ. En estos pacientes prevalecen los signos y síntomas de Artritis reumatoide y el compromiso renal está asociado con Lupus. Objetivo: Describir las características clínicas y hallazgos histopatológicos renales en pacientes con Ruphus. Materiales y métodos: Reporte de Casos detectados en forma retrospectiva en el Hospital Nacional Arzobispo Loayza, entre los años 2003 al 2009 y sus biopsias renales. Resultados: Hubo 7 pacientes con ruphus, la mediana de edad fue 40 años, una mediana de tiempo de evolución de 2 años. Los hallazgos histopatológicos renales relevantes fueron Glomerulonefritis lúpica tipo IV en 5 pacientes; proteinuria entre 288 a 2560 mg/24 horas y títulos de Factor Reumatoide entre 14 a 34,6 UI/L. Conclusiones: Los pacientes reportados a diferencia de la literatura fueron de mayor edad, con compromiso renal moderado a severo relacionado a Lupus.

Up to 10% of patients with Systemic Lupus Erythematosus have a joint condition similar to that of rheumatoid arthritis, which is called ôRhupusõ. These patients predominantly have signs and symptoms of rheumatoid arthritis and kidney damage associated with Lupus. Objectives: To describe the clinical characteristics and the renal histopathological findings in patients with rhupus. Materials and methods: Retrospective review of case reports and kidney biopsies from patients seen from 2003 to 2009 in Arzobispo Loayza National Hospital. Results: There were 7 patients with rhupus; their median age was 40 years, the median time of disease progression was 2 years. Most relevant renal histopathological findings were type IV glomerulonephritis in 5 patients; proteinuria between 288 to 2560 mg/24 hours and rheumatoid factor titers between14 to 34.6 UI/L were also found. Conclusions: Patients were older compared to others previously reported previously in the literature. Lupus-related renal damage was moderateto severe.

Nephrogenic systemic fibrosis following acute kidney injury and exposure to gadolinium.

Nephrogenic systemic fibrosis (NSF) is a scleroderma-like systemic fibrosing condition of unknown etiology described in patients with renal insufficiency. Gadolinium exposure has been strongly associated with the development of NSF though the mechanism of such injury is not known. There are only few reported cases of NSF in the setting of acute renal failure and fewer reported cases where skin lesions developed after kidney function had returned to normal. We report a case of NSF in a young Hispanic woman with lupus nephritis but normal creatinine, who received gadolinium during a brief episode of prerenal acute kidney injury not requiring dialysis, secondary to sepsis.

Clinical features and outcome of systemic lupus erythematosus.

We report the clinical profile, treatment and outcome of systemic lupus erythematosus in 70 patients between the age of 4-15 years. Fever (94.2%), arthritis (65.7%) and malar rash (57.1%) were the chief extra-renal manifestations. The ESR was raised in 98.5% patients, anemia was seen in 60% and direct Coombs test was positive in 58.3%. Antinuclear antibody was positive in all; anti-double stranded DNA antibody and low C3 levels were seen in 77.1% and 80%, respectively. Renal involvement was noted in 77.1% and included proteinuria (53%), hematuria (42.8%), hypertension (18.5%) and elevated serum creatinine (8.6%). Renal histology showed class I nephritis in 3.7%, class II in 44.4%, class III in 4.3%, class IV in 44.4% and class V in 1.8%. On follow up 18.8 months later, 70% patients were in remission, 7.5% had active disease and 7.5% died. The characteristics of childhood lupus erytematosus were similar to those previously reported. The outcome was favorable in most cases.

Associação entre anticorpos antifosfolípides e fator reumatóide empacientes com nefrite lúpica: um estudo de 187 casos / Associatiom between antiphospholipid antibodies and rheumatoid factor in patients with Lupus Nephrits: a study of 187 cases

Introdução: Nefrite é uma manifestação freqüente no lúpus eritematoso sistêmico (LES), tendo uma prevalência de 40 a 75%. Embora esteja associada com a ocorrência de anticorpo anti-DNA, outros autoanticorpos podem estar ligados ao seu aparecimento taiscomo o fator reumatóide (FR) e os anticorpos antifospholípides (aPl).Objetivos: verificar a prevalência de nefrite lúpica local e sua associação com o a presença do FR e aPls. Métodos: Revisaram-se 187 prontuários depacientes com LES para presença de nefrite, FR e aPl . Resultados: Encontrou-se uma prevalência de 35,3% de glomerulonefrite. Houve correlação negativa entre nefrite e FR (p=0.001). Não se encontrou associação entre aparecimento de aPl e nefrite, já queo número de pacientes com glomerulonefrite que apresentaram esses testes positivos foram de 8 (32%)em 59; 5 (27,7%) em 58 e 6 (46,1%) em 29, respectivamente. Conclusão: Existe uma prevalência de 35% denefrite na população local de LES a qual tem uma associação negativa com aparecimento do FR. Presença de aPls não influíram no aparecimento da nefrite lúpica.

Summary: Nephritis is a frequent manifestation of systemic lupus erythematosus (SLE), with an averageof 40 to 75%. It’s associated with the presence of anti-DNA antibodies;other antibodies may also be involved,such as rheumatoid factor (RF), and antiphospholipid antibodies (aPl).Objectives: to verify the prevalence of lupus nephritis and its association with RF and aPl antibodies. Methods: The charts of 187 patients with SLE werereviewed for the presence of nephritis, RF and aPl. Results: A prevalence of 35.3% of nephritis was found. There was a negative correlation betweennephritis and presence of rheumatoid factor (p=0,001). No association between the presence of aPl and nephritis was found, since the number of patients with glomerulonephritis that were positive for these tests wasof 8 (32%) in 59, 5 (27,77%) in 58 and 6 (46,1%) in 29, respectively.Conclusion: There is a prevalence ofglomerulonephritis of 35% in the local SLE population, which has a negative association with rheumatoid factorpresence. Antiphospholipid antibodies aren’t associated to the occurrence of lupus nephritis.

Renal histological findings in adults in Jamaica / Hallazgos histológicos renales en adultos en Jamaica

BACKGROUND: In 2006, it was reported that Focal and Segmental Glomerulosclerosis (FSGS), Minimal Change Disease (MCD) and Membranous Glomerulonephritis (MGN) were the commonest primary glomerular diseases identified from percutaneous kidney biopsies done in Jamaica for that year (n = 76). The sample size was thought to be small and might have affected the reported findings. So a threeyear review of percutaneous kidney biopsies in Jamaica was carried out. METHODS: Histology reports and clinical data were reviewed for percutaneous kidney biopsies performed from January 2005 to December 2007. Demographic data (age, gender), laboratory investigations such as serum urea, serum creatinine, proteinuria, haematuria, 24-hour urinary protein, and creatinine clearance, and clinical diagnosis were collected from the histology requisition form. RESULTS: There was a total of 224 native kidney biopsies performed. There were 91 males (40.6%) and 133 females (59.4%). Age distribution showed a total number of 25 paediatric cases (11.2%) and 199 adult cases (88.8%). Proteinuria was present in 171 cases (76.3%) and haematuria in 86 cases (38.4%). Of the total biopsies done, 78 cases (39.2%) had primary glomerular diseases, 110 cases (55.3%) had secondary glomerular diseases and 11 (5.5%) biopsies were reported as either normal or inadequate for histological diagnosis. The most common reasons indicated for percutaneous kidney biopsy were proteinuria, haematuria and staging of lupus nephritis. Most common histological findings for primary glomerular disease after percutaneous kidney biopsy were FSGS (n = 34), MGN (n = 15) and MCD (n = 12). In secondary glomerular diseases (n = 110), there were more females (70.8%) than males. Systemic lupus erythematosus was present in 63.3%. Histology of lupus nephritis according to the International Society of Nephrologists classification shows Membranous Lupus Nephritis [MLN] (40.2%), Diffuse Lupus Nephritis [DLN] (19.5%) and Minimal Mesangial Lupus Nephritis [MMLN] (14.3%) as the common histological types. CONCLUSIONS: The most common histological finding for primary glomerular disease following percutaneous kidney biopsy was FSGS, MCD and MGN. Membranous Lupus Nephritis was the commonest histological type for lupus nephritis in this series.

ANTECEDENTES: En 2006, se reportó que la Glomeruloesclerosis Segmentaria y Focal (GESF), la Enfermedad de Cambios Mínimos (ECM) y la Glomerulonefritis Membranosa (GNM) fueron las enfermedades glomerulares primarias más comunes identificadas a partir de las biopsias renales percutáneas realizadas en Jamaica ese año (n = 76). El tamaño de la muestra se consideró pequeño y pudo haber afectado los hallazgos reportados. De manera que se realizó un examen de tres años, de las biopsias renales percutáneas en Jamaica. MÉTODOS: Se revisaron los reportes de histología y los datos clínicos correspondientes a las biopsias renales percutáneas realizadas desde enero de 2005 a diciembre de 2007. RESULTADOS: Hubo un total de 224 biopsias de riñón nativo. Se realizaron 74, 78 y 72 biopsias renales en 2005, 2006 y 2007 respectivamente. Hubo 91 varones (40.6%) y 133 hembras (59.4%). La distribución por edades mostró un total de 25 casos pediátricos (11.2%) y 119 casos de adultos (88.8%). La proteinuria estuvo presente en 171 casos (76.3%) y la hematuria en 86 casos (38.4%). Del total de biopsias realizadas, 78 casos (39.2%) tenían enfermedades glomerulares primarias, 110 casos (55.3%) tenían enfermedades glomerulares secundarias y 11 (5.5%) biopsias fueron reportadas como normales, o como inadecuadas para el diagnóstico histológico. Las razones más comunes señaladas para la biopsia renal percutánea fueron la proteinuria, la hematuria y la estadificación de la nefritis por lupuso nefritis lúpica. Los hallazgos histológicos más comunes para la enfermedad glomerular primaria tras la biopsia renal percutánea fueron GESF (n = 34), GNM (n = 15) y ECM (n = 12). En relación con las enfermedades glomerulares secundarias (n = 110), hubo más hembras (70.8%) que varones. El lupus eritematoso sistémico estuvo presente en 63.3%. De acuerdo con la clasificación de la Sociedad Internacional de Nefrología, la histología de la nefritis por lupus muestra la nefritis lúpica membranosa (NLM) [40.2%], la nefritis lúpica difusa (NLD) [19.5%], y la nefritis lúpica mesangial mínima (NLMM) [14.3%], como los tipos histológicos más comunes. CONCLUSIÓN: Los hallazgos histológicos más comunes para la enfermedad glomerular primaria tras la biopsia renal percutánea, fueron GESF, ECM y GNM. La nefritis lúpica membranosa fue el tipo de histología más común para la nefritis por lupus en esta serie.

Renal biopsy in systemic lupus patients with low level proteinuria, and its relation to urinary adhesive molecules

To assess whether systemic lupus [SLE] patients with levels of proteinuria <1000 mg/24h should be routinely biopsied, or we can depend on urinary adhesion molecules [ICAM-1 and VCAM-1] as markers for the severity of lupus nephritis [LN]. This study included 30 SLE patients with proteinuria <1000 mg/24h, and 20 SLE patients without proteinuria. Twenty healthy control subjects were also included. Basic laboratory parameter, RF, ANA, Anti-dsDNA, Complement C3 and C4, Urinary ICAM-1 and VCAM-1 and estimation urinary total proteins/24 hours were performed for each subject. Renal biopsy was also done in the indicated cases. Twenty three of thirty biopsies were diagnostic of lupus nephritis: 3 mild nephrosclerosis, 6 mesangial proliferation, 8 focal proliferative, 5 diffuse proliferative, and 1 membranous lupus nephritis. The levels of proteinuria were 94.5 +/- 22 mg/24h, 119 +/- 33 mg/24h and 725 +/- 180 mg/24h for control, patients without proteinuria and patients with proteinuria respectively. No statistical differences could be detected as regards to C3, C4, urinary ICAM-1 and urinary VCAM-1. Patients were reclassified according to the severity of renal histopathological changes into mildly and severely affected groups. We found no statistical differences between both groups as regards C3 and C4, but there were high statistical differences in urinary ICAM-1 and urinary VCAM-1 [P value <0.0005]. Our findings suggest that renal biopsy should be performed in these patients in the presence of new onset or rising proteinuria to enable prompt diagnosis of LN and initiation of treatment earlier in the course of the disease

Nefropatía C1q en un paciente de 17 años: caso clínico / C1q nephropathy: report ofone case

Clq nephropathy (Clq N) is an infrequent disease and only about 100 cases have been reponed. It is defined by a pattern of immunofluorescense (IF) with dominant or co-dominant complement Clq with electrondense deposits in the mesangium, without clinical or serological features of Lupus Nephritis. The most common histopathological findings of ClqN are focal segmental glomerulosclerosis and Minimal Change Disease. We repon a 17 year-old male patient with an isolated selective proteinuría found in a routine study. He had normal renal function and uriñe culture was negative. Serum lipids, liver enzymes an complement were all normal. Serum antinuclear and anti-DNA antibodies, antineutrophil cytoplasmic antibodies (ANCA), HIV, Hepatitis B and C serology, were negative. Renal and abdominal ultrasonography was normal. The histopathological study revealed segmental glomerular sderosis, modérate increase of mesangial matrix, Bowmann capsule adhesions and fucsinophil deposits in mesangium. The IF was positive (dominant) for Clq (+++) and IgA, IgG, IgM, C3++, all of them with a granular mesangial distribution. Ultrastructural findings were pedicelar effacement and paramesangial electrondense deposits. Tubular reticular inclusions (TRI) were not found. Remission of proteinuría was reached after 18 months of treatment with enalapril and losartan. The patient remains with normal renal function. Clinical findings, negative serology for Lupus, light microscopy IFwith dominant positivity for Clq, absence of TRI and paramesangial electrondense deposits in electron microscopy lead us to the diagnosis of ClqN. A poor response to steroid therapy was described in ClqN. Thus it was worthwhile to differentiate it from lupus nephritis, that is responsive to steroids.

Prediction of tubulo-interstitial injury by Doppler ultrasound in glomerular diseases: value of resistive and atrophic indices.

BACKGROUND: Doppler ultrasound is increasingly used in Nephrology for diagnosis of renovascular hypertension and evaluation of allograft dysfunction. However, its utility in glomerular disease remains controversial. OBJECTIVES: Using Doppler Ultrasound, we prospectively tested the role of resistive and atrophic indices in predicting tubulointerstitial lesions in patients with glomerular disease as demonstrated by renal biopsy. METHODS: Seventy one patients with primary or secondary glomerular diseases were examined by Doppler ultrasonography immediately before renalbiopsy. The resistive and atrophic indices (RI & AI) were calculated and compared with histologic changes in biopsy specimen. RESULTS: Receiver Operator Characteristics analysis showed RI of 0.60 as an optimal value for discriminating tubulointerstitial changes with sensitivity of 82.7% and specificity of 92%. An AI of 0.65 was shown to be optimal for discriminating tubulointerstitial injury with sensitivity of 69.2% and specificity of 85%. The combination of the two indices had not been found to be superior to either index alone. There was a significant correlation between atrophic and resistive indices. (r=0.358, p< 0.01). It was observed that older age, smoking, elevated AI and RI, low GFR, high serum cholesterol and Hypertension were found to be significantly associated with the presence of tubulointerstitial injury in the univariate analysis whereas only elevated AI and RI were found to predict tubulointerstitial injury in multivariate analysis. CONCLUSION: Measurement of RI by Doppler ultrasound can be considered as a supplementary diagnostic tool in glomerular diseases to predict the severity of tubulointerstitial injury.

Lupus nephritis in children

To determine the clinicopathological pattern of lupus nephritis in paediatric nephrology patients. Case series study. The department of paediatric nephrology at the Children's Hospital and Institute of Child Health, Lahore, Pakistan, over a period of five years from January 2001 to December 2005. Twenty six patients upto the age 16 years of either gender, with a mean age of 12.4 _ 1.90 years having primary SLE with renal involvement in the form of oedema, hypertension, haematuria and proteinuria were included. Twenty one were females. Percutaneous renal biopsy was performed. Histological lesion was classified according to WHO classification. Patients were treated with immunosuppressive therapy and their clinical course was followed for at least one year. The mean duration of follow up was 1.77 years. Renal involvement was seen in 92.30% within 2 years of the onset of primary disease. Diffuse proliferative glomerulonephritis was the commonest histological lesion [n=14] followed by membranous nephropathy [n=6]. The commonest clinical manifestation was oedema [80.76%] followed by hypertension [46.15%]. Proteinuria was present in 100% of cases, haematuria in 38.46% and azotemia in 19.33% of patients. Nephrotic range proteinuria was more common in class III and IV, while azotemia was observed only in class IV. The disease was well controlled in 73.07%, relapse was seen in 3.8% of patients, 15.38% died of infections and uremic encephalopathy while 7.69% were lost to follow-up. Diffuse proliferative glomerulonephritis is the commonest histological lesion in our set-up. Renal involvement is mostly seen within first two years of the primary disease which can be controlled satisfactorily with immunosuppressive therapy

role of anticardiolipin and anti-B2 glycoprotein antibodies in clinical complication of lupus nephritis

Although there was confliction regarding the association of raised anticardiolipin antibodies [ACL] level with renal disease in systemic lupus erythematosus [SLE], the role of B2glycoprotein as a cofactor for ACL binding is established.The presence of ACL and anti-B2GPI may be directly involved in pathogenesis of antiphospholipid antibodies associated symptoms like recurrent fetal loss, thrombocytopenia and thrombosis. To study the possible association between some auto antibodies with the most common clinical complication of disease. The study was conducted on 25 patients with lupus nephritis, attended the renal clinic in specialized surgical hospital/ medical city /Baghdad, 25 lupus patients without nephritis and 25 healthy controls. Enzyme linked immunsorbant assay was used for detection for ACL, anti-B2GP. Although there was no significant difference in mean concentrations of ACL and anti- B2GP between lupus nephritis and without nephritis [P>0.05], lupus nephritis patients were more likely positive for ACL. Positivity of 100% For ACL and anti-_B2GPwere detected in thrombotic complication, in fetal loss complication patients more likely to be positive for ACL [75%], anti-B2GP [50%], in thrombocytopenia positivity of ACL and anti-B2GP [75%]. There was no association between anti-B2GP and renal disease and presence of both ACL and anti-B2GP carry higher risk for thrombosis and recurrent fetal loss

Correlación entre el daño tubulointersticial y vascular en la nefritis lúpica con algunas variables clínicas / Correlation between the tubulointerstitial and vascular damage in lupus nephritis with some clinical variables

Las lesiones tubulointersticiales (TI) y vasculares (VS) son frecuentes en la nefritis lúpica (NL) a pesar de no ser consideradas en la clasificación de la OMS de 1995. Se revisaron 339 biopsias renales para correlacionar ciertas variables clínicas con las lesiones del TI y los vasos y saber la frecuencia con la que se producen. Se determinó la frecuencia del daño TI 101/29,8 por ciento y VS 65/19,2 por ciento. Se tuvo en cuenta además la incidencia de las lesiones TI en los distintos tipos de glomerulonefritis lúpica: 0 en la tipo I; 7/9,8 por ciento en la II; 8/15,7 por ciento en la III; 65/50 por ciento en la IV; 2/6,3 por ciento en la V y 16/100 por ciento en la VI. Quedó evidenciado que el daño TI y VS fue expresión de severidad clínica, pues se acompañó de mayor frecuencia de hipertensión 83/82 por ciento y 64/97 por ciento, que cuando no existieron estas lesiones 128/58 por ciento, mayor tiempo medio de evolución de la enfermedad: 3,4; 2,8 y 1,89 años y cifras de creatinina plasmática 189,1; 172,1 y 134,5 mmol/L para el daño TI, VS y la ausencia de estos, respectivamente. La proteinuria fue mayor en presencia de lesión TI, 3,4 g/L y menor en los pacientes con daño VS, 1,89, lo contrario a la incidencia de hematuria, 65/100 por ciento VS y 48/47,5 por ciento TI. En los pacientes sin lesión en estas estructuras los resultados fueron: 2,02 g/L para la proteinuria y 143/61 por ciento para la hematuria

Terapia de rescate con micofenolato mofetil en pacientes con nefritis lúpica / Rescue therapy in lupus nephritis patients with micophenolato mofetil

La terapia a largo plazo con ciclofosfamida combinada con esteroides mejora la sobrevida renal en pacientes con Nefritis Lúpica Proliferativa, pero con efectos tóxicos considerables. En años recientes, el Micofenolato Mofetil (MMF) un inmunosupresor usado en trasplante, parece ser efectivo en casos selectos de Nefritis Lúpica. Métodos: Describiremos 6 pacientes con Nefritis Lúpica clase IV y V según OMS que por distintas razones debieron ser tratados con MMF como droga de rescate. El mismo estabilizó la función renal. Controló la actividad del LES y permitió la disminución o suspensión del corticoide. Resultados: En tres casos se logró remisión completa, dos presentaron remisión parcial y tuvimos un fracaso por plaquetopenia y leucopenia severa con sepsis grave a punto de partida de una neumopatía; en este paciente se suspendió el tratamiento. Uno de ellos presentó un herpes zóster que se trató con Aciclovir y la suspensión transitoria de MMF. Conclusión: EL MMF fue efectivo para conseguir remisiones en la NL y mantener inactivo el LES, por lo que se lo debiera considerar como terapia de rescate, o bien para tratamiento de mantenimiento luego de la inducción con ciclofosfamida, o directamente como terapia de inducción en aquellos pacientes donde la fertilidad es un factor importante. Seguimientos a largo plazo de las pacientes son necesarios para evaluar su efectividad en la sobreviva renal, como ya ha sido demostrado con la Ciclofosfamida.

Long term cyclophosfamide combinated steroids therapy improves renal survival in patients with Proliferate Lupus Nephritis, with considerable toxic effects. In lately years MMF, a immune suppressor used in transplant, seems to be effective in selected cases of Lupus Nephritis. Methods: We will describe six patients with Lupus Nephritis class IV and V (OMS Classification). that what different causes they must be treated with MMF like rescue drug. This stabilizes the renal function. controls LES activity and allows reductions or end of corticoids. Results: In tree cases we achievement total remission, two show partial remission and we had a fail because plaquetopenia and severe leucopenia with serious sepsis to give rise to neumopaty; this patient broke off the treatment. One presented Zoster...

Nefritis lúpica en la Clínica San Pedro Claver 1998-2003. Hallazgos clínicos y correlación clínico patológica / Lupus nephritis in Clínica San Pedro Claver 1998-2003

Objetivo. Conocer el comportamiento de la nefritis lúpica en nuestra población. Determinar la frecuencia de las principales variables clínicas que acompañan esta patología. Búsqueda de tendencias entre el tipo histológico de la nefritis con sus hallazgos clínicos. Métodos. Estudio descriptivo realizado en la Unidad Renal de la Clínica San Pedro Claver de Bogotá (IV Nivel), en el período comprendido entre marzo de 1998 y septiembre de 2003. Se evaluaron 145 pacientes quienes asistieron a la consulta ambulatoria remitidos para valoración de compromiso renal con diagnóstico de LES. Resultados. Se evaluaron 145 casos. Fueron excluidos 7 casos por no cumplir con cuatro de los 11 criterios de la ACR para el diagnóstico de LES. Predominó el género femenino, con 130 casos (94,2 por ciento), sobre el masculino con 8 casos (5,8 por ciento). La edad promedio de diagnóstico de LES fue de 29,6 años. La distribución por grupos etáreos mostró que los pacientes con edades entre 20 y 50 años representan el 85 por ciento. Se encontraron al 45 por ciento de los pacientes hipertensos y con proteinuria el 58 por ciento. En el 68 por ciento de los casos se evideció disminución de la tasa de filtración glomerular. Se realizó biopsia en el 51 por ciento de los pacientes. Según la clasificación de la OMS (1996) se observó del tipo I el 1 por ciento, del tipo II y tipo III 17 por ciento cada una, tipo IV el 48 por ciento y del tipo V el 10 por ciento. No se observó ninguna tendencia entre el tipo de biopsia y el grado de compromiso renal, ni con la progresión a insuficiencia renal crónica terminal. Discusión. Los resultados mostraron una distribución por género, grupo etáreo y biopsia, similar a los informados en la literatura. El número de pacientes hipertensos, con presencia de proteinuria y disminución en la tasa de filtración glomerular fueron mayores comparados con las grandes series internacionales. No se observó relación entre el tipo de biopsia OMS encontrado y los pacientes que progresaron a insuficiencia renal crónica terminal

Curso clínico de la nefropatía membranosa lúpica pura / Long-Term outcome of type V lupus membranous glomerulonephritis

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.

Glomerulonefritis lupica: relación entre severidad de la nefropatía y variables funcionales renales / Lupus Nephritis: correlation between severity of nephropaty and renal functional parameters

Para investigar la diferencia de la proteinuria (Pr) de acuerdo a la severidad de la nefropatía y a su posible origen inmunológico o hemodinámico, se investigó el comportamiento de lagunos parámetros funcionales renales en pacientes con Lupus Eritematoso Sistémico y Nefropatía (GN-LES) leve o severa antes y después de la sobrecarga de proteínas de alto valor biológico (PAVB). Se estudiaron 23 pacientes con GN-LES y 10 controles sanos. A pacientes y controles se les practicó ecosonograma renal y perfil inmunológico para lupus eritematoso sistémico; Pr cuantitativa, tasa de filtración glomerular (TFG) medida por la depuración de creatinina (DCr), excreción fraccional de sodio (FeNa) y reserva funcional renal (RFR) fueron determinadas en condiciones basales y postcarga. Los 23 pacientes con GN-LES fueron sometidos a biopsia renal y sus lesiones clasificadas en leves y (Clases IIa y IIb; n=5) y severas (clases III,IV, y V; n=18) de acuerdo a los criterios de la OMS. La diferencia en la magnitud de la Pr entre ambos grupos fue marcada clínicamente, sin significancia estadística (p=0.110). El grupo con GN-LES leve presentó una Pr basal y postcarga de 0.281 ± g/24 horas y 0.480 ± 0.306 g/24 horas respectivamente, (p=0.05). La TFG en las formas leves varió postsobrecarga (86.84 ± 36.98 ml/min vs. 140.86 ± 38.35 ml/min; p=0.036), indicando RFR normal. En las formas severas la TFG se modificó poco postcarga (79.46 ± 42.48 ml/min vs. 89.63 ± 65.13 ml/min) p=0.58, representando una RFR reducida o ausente. La FeNa fue normal en ambos grupos tanto en condiciones basales como postcarga. Los parámetros funcionales renales evaluados no se correlacionaron directamente con la actividad y cronicidad de la GN-LES, ni con el posible origen inmunológico o hemodinámico de la proteinuria. En pacientes con GN-LES la sobrecarga con PAVB, mostró ser un método útil en la determinación de la RFR y en la detección de daño parenquimatoso renal precoz

Compromiso cardiovascular en niños con lupus eritematoso sistémico / Cardiovascular involvement in children with systemic lupus erythematosis

El Lupus Eritematoso Sistémico (LES) es una enfermedad poco frecuente en niños y ocupa el segundo lugar entre las mesenquimopatías infantiles. La expresión clínica en niños es de mayor severidad y debe tenerse presente tanto por su elevada morbimortalidad como también por su potencial incapacitante. El compromiso cardiovascular se estima en 30 a 40 por ciento, pudiendo ser primario o secundario a la terapia esteroidal. Las manifestaciones clínicas son variadas, constituyendo la pericarditis y miocarditis las complicaciones más frecuentes. Con el propósito de dar a conocer el compromiso cardiovascular existente en esta enfermedad se describen tres casos clínicos con diagnóstico de LES, en quienes el compromiso cardiovascular fue la manifestación clínica más importante