Comparison of lupus patients with early and late onset nephritis: a study in 71 patients from a single referral center

Abstract Background: Nephritis occurs frequently in systemic lupus erythematosus (SLE) and may worsen disease morbidity and mortality. Knowing all characteristics of this manifestation helps to a prompt recognition and treatment. Aim: To compare the differences in clinical data, serological profile and treatment response of nephritis of early and late onset. Methods: Retrospective study of 71 SLE patients with biopsy proven nephritis divided in early nephritis group (diagnosis of nephritis in the first 5 years of the disease) and late nephritis (diagnosis of nephritis after 5 years). Epidemiological, serological, clinical and treatment data were collected from charts and compared. Results: In this sample, 70. 4% had early onset nephritis and 29.6% had late onset. No differences were noted in epidemiological, clinical, serological profile, SLICC and SLEDAI, except that late onset nephritis patients were older at nephritis diagnosis (p = 0.01). Regarding renal biopsy classification, C3 and C4 levels, serum creatinine, 24 h proteinuria and response rate to treatment the two groups were similar (p = NS). Patients with early onset had lower levels of hemoglobin at nephritis onset than those of late onset (p = 0.02). Conclusions: Most of SLE patients had nephritis in the first 5 years of disease. No major differences were noted when disease profile or treatment outcome of early and late onset nephritis were compared.(AU)

Henoch-Schönlein purpura nephritis: initial risk factors and outcomes in a Latin American tertiary center.

The objective of this study was to evaluate prevalence, initial risk factors, and outcomes in Henoch-Schönlein purpura nephritis (HSPN) patients in Latin America. Two hundred ninety-six patients (validated EULAR/PRINTO/PRES HSP criteria) were assessed by demographic data, clinical/laboratorial involvements, and treatments in the first 3 months after diagnosis. They were followed-up in a Latin American tertiary center and were divided in two groups: with and without nephritis. Persistent non-nephrotic proteinuria, nephrotic proteinuria, and acute/chronic kidney injury were also systematically evaluated at 1, 5, 10, and 15 years after diagnosis. HSPN was evidenced in 139/296 (47%) in the first 3 months. The median age at diagnosis was significantly higher in HSPN patients compared without renal involvement [6.6 (1.5-17.7) vs. 5.7 (0.9-13.5) years, p = 0.022]. The frequencies of persistent purpura (31 vs. 10%, p < 0.0001), recurrent abdominal pain (16 vs. 7%, p = 0.011), gastrointestinal bleeding (25 vs. 10%, p < 0.0001), and corticosteroid use (54 vs. 41%, p = 0.023) were significantly higher in the former group. Logistic regression demonstrated that the independent variables associated with HSNP were persistent purpura (OR = 3.601; 95% CI (1.605-8.079); p = 0.002) and gastrointestinal bleeding (OR = 2.991; 95% CI (1.245-7.183); p = 0.014). Further analysis of patients without HSPN in the first 3 months revealed that 29/118 (25%) had persistent non-nephrotic proteinuria and/or hematuria in 1 year, 19/61 (31%) in 5 years, 6/17 (35%) in 10 years and 4/6 (67%) in 15 years after diagnosis. None of them had chronic kidney injury or were submitted to renal replacement therapy. The present study observed HSPN in almost one half of patients in the first months of disease, and HSPN was associated with persistent purpura and gastrointestinal bleeding. One fourth of patients had nephritis only evidenced during follow-up without severe renal manifestations.

Outcomes of 847 childhood-onset systemic lupus erythematosus patients in three age groups.

Objective The objective of this study was to assess outcomes of childhood systemic lupus erythematosus (cSLE) in three different age groups evaluated at last visit: group A early-onset disease (<6 years), group B school age (≥6 and <12 years) and group C adolescent (≥12 and <18 years). Methods An observational cohort study was performed in ten pediatric rheumatology centers, including 847 cSLE patients. Results Group A had 39 (4%), B 395 (47%) and C 413 (49%). Median disease duration was significantly higher in group A compared to groups B and C (8.3 (0.1-23.4) vs 6.2 (0-17) vs 3.3 (0-14.6) years, p < 0.0001). The median Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR-DI) (0 (0-9) vs 0 (0-6) vs 0 (0-7), p = 0.065) was comparable in the three groups. Further analysis of organ/system damage revealed that frequencies of neuropsychiatric (21% vs 10% vs 7%, p = 0.007), skin (10% vs 1% vs 3%, p = 0.002) and peripheral vascular involvements (5% vs 3% vs 0.3%, p = 0.008) were more often observed in group A compared to groups B and C. Frequencies of severe cumulative lupus manifestations such as nephritis, thrombocytopenia, and autoimmune hemolytic anemia were similar in all groups ( p > 0.05). Mortality rate was significantly higher in group A compared to groups B and C (15% vs 10% vs 6%, p = 0.028). Out of 69 deaths, 33/69 (48%) occurred within the first two years after diagnosis. Infections accounted for 54/69 (78%) of the deaths and 38/54 (70%) had concomitant disease activity. Conclusions This large multicenter study provided evidence that early-onset cSLE group had distinct outcomes. This group was characterized by higher mortality rate and neuropsychiatric/vascular/skin organ damage in spite of comparable frequencies of severe cumulative lupus manifestations. We also identified that overall death in cSLE patients was an early event mainly attributed to infection associated with disease activity.

Nefritis aguda focal: caso clínico / Focal acute nephritis: a case report

Focal acute nephritis (FAN) or acute lobar nephronia is a rare clinical picture characterized by an infection localized in the kidney, with an inflammatory mass without liquefaction. Of variable clinical manifestations, diagnosis is achieved through CT scanning. Histologically, unlike acute pyelonephritis, it does not present a diffuse infíltrate. Objective: Case report of FAN in a pediatric patient. Case Report: Ten year old male complaining of abdominal pain, presents painful percussion in the right lumbar fossa. Urinary analysis and culture were negative, renal sonogram was negative. Abdominal CT sean showed múltiple hypodense renal foci. He responded well to cephotaxim, being discharged in the third day of hospitalization with completion of treatment as outpatient. Differential diagnosis with Acute Pyelonephritis is very important, as it requires controlled and opportune treatment to prevent renal absceses. Diagnosis of this pathology must be pursued despite a normal UA.

La nefritis aguda focal o nefronia lobar aguda constituye un cuadro poco común caracterizado por una infección localizada en el riñon, la que corresponde a una masa inflamatoria sin licuefacción. Posee una clínica variable, siendo la tomograña computada (TAC) la prueba más sensible y específica para el diagnóstico de esta enfermedad. Esta patología se diferencia histológicamente de la pielonefritis aguda por no presentar un infiltrado inflamatorio difuso. Objetivo: presentar un caso de nefronia aguda multifocal en un paciente pediátrico. Caso clínico: Escolar de 10 años que consultó por dolor abdominal, al examen destacaba la presencia de percusión dolorosa en fosa lumbar. Los exámenes de orina y urocultivo fueron negativos. Al ingreso no se detectó cambios renales ecográficamente evidenciables. Se realizó un TAC de abdomen que mostraba múltiples focos renales hipodensos. Respondió favorablemente a terapia antibiótica con cefotaxima siendo dado de alta al tercer día, completando terapia en forma ambulatoria. La diferenciación de este cuadro de otros procesos renales como la pielonefritis aguda (PNA) es muy importante, ya que precisa un tratamiento oportuno y controlado por el riesgo de evolucionar a absceso renal. El diagnóstico de esta patología debe ser buscado a pesar de contar con exámenes de orina negativos.

[BK virus infection in a pediatric renal transplant recipient]. / Infección por virus BK en paciente pediátrico trasplantado renal.

BK Human Polyomavirus causes an asymptomatic primary infection in children, then establishing latency mainly in the urinary tratt. Viral reactivation can lead to renal pathology in individuals with impaired cellular immune response. This is particularly important in pediatric transplant recipients, who can suffer a primary infection when immunosupressed. We followed up the case of a 5 years old patient who received a renal transplant in October 2003, and presented damaged graft 45 days after the intervention. The patient suffered 3 episodes of renal function failure between October 2003 and June 2004. Blood, urine, renal biopsy and lymphocele liquid samples were analyzed. A differential diagnosis between acute rejection and infectious causes was established by testing for BK, CMV and ADV viruses, and the cytological study of renal tissue. Laboratory findings together with clinical signs suggest the patient was infected by BK virus. As a final consideration, the great importance of differentiating between acute rejection and BK infection is emphasized, since immunosuppressant management is opposite in each case.

Diagnósticos de enfermería identificados en un niño con el síndrome de Denys Drash / Nursing diagnoses identified in a child with the Denys Drash syndrome

Se realiza el estudio desde el punto de vista de enfermería de un paciente con el síndrome de Denys Drash. Fueron seleccionados los principales problemas presentados por el paciente durante su hospitalización y se consultaron distintos diagnósticos de la NANDA. Teniendo en cuenta que la familia tenía dificultades para adaptarse a esta nueva situación se seleccionaron 2 que dieron respuesta satisfactoria, hasta el momento de la culminación del protocolo. El método utilizado durante la investigación fue observación directa en su modalidad de participante, se consultó la historia clínica y se seleccionaron los datos más interesantes. Se revisaron los diagnósticos, expectativas y órdenes impartidas por el personal de enfermería. Del trabajo realizado se obtuvieron resultados satisfactorios, siempre recordando la entidad y su pronóstico evolutivo, después de finalizada la investigación se cumplió el pronóstico descrito (AU)

Nefritis colagena / Collagen nephritis

En los últimos años se ha descripto un nuevo tipo de glomerulopatía primaria, denomminada nefritis colágena, caracterizada por la acumulación masiva de colágeno fibrilar en mesangio y membrana basal glomerular sin asociación con patología osteoarticular. Se comunica aquí el primer caso en nuestro medio. Se trata de una paciente de 26 años con síndrome nefrótico y progressiva caída del filtrado cuya biopsia renal mostró una esclerosis focal y segmentaria en la microscopia óptica. La evaluación ultraestructural reveló acúmulos en membrana basal y mesangio de colágeno fibrilar que permitió el diagnóstico de esta entidad.

Nefritis colagena / Collagen nephritis

En los últimos años se ha descripto un nuevo tipo de glomerulopatía primaria, denomminada nefritis colágena, caracterizada por la acumulación masiva de colágeno fibrilar en mesangio y membrana basal glomerular sin asociación con patología osteoarticular. Se comunica aquí el primer caso en nuestro medio. Se trata de una paciente de 26 años con síndrome nefrótico y progressiva caída del filtrado cuya biopsia renal mostró una esclerosis focal y segmentaria en la microscopia óptica. La evaluación ultraestructural reveló acúmulos en membrana basal y mesangio de colágeno fibrilar que permitió el diagnóstico de esta entidad. (AU)

Focal bacterial nephritis (lobar nephronia) in children.

We report 13 patients with 16 episodes of acute lobar nephronia diagnosed in a prospective study that was conducted among 210 hospitalized children with urinary tract infection. In 30 episodes of urinary tract infection, a hypoechogenic or hyperechogenic lesion was found. Twenty patients underwent computed tomography, and in 16 of them acute lobar nephronia was diagnosed. Evolution to renal abscess occurred in 25%. Prolonged intravenous antibiotic treatment was sufficient in all cases.

[Nephropathy in Schönlein-Henoch purpura]. / Nefropatía en la púrpura de Henoch Schonlein.

Over one hundred patients diagnosed with vascular purpura at the National Institute of Pediatrics between 1979 and 1988 were retrospectively studied. Frequency was similar for both sexes. 82% were between the ages of six months and 11 years old. The extrarenal clinical manifestations were: petechias in 98%; abdominal pain 78% arthralgias 45%; melena 39%; and arthritis in 19% of the cases, 49 patients had nephropathy of different degrees and they did differently, 32 had hematuria with or without proteinuria within a nephrotic range, seven suffered from a nephrotic syndrome, one with a nephritic syndrome and nine others had a combination of two or three syndromes (nephritic/nephrotic/renal failure) with an unfavorable evolution towards terminal renal failure in eight of them; on the other hand, this was not seen in the remaining patients. Thirteen renal biopsies were taken from patients with more severe clinical manifestations, finding in them mesangial proliferation or endo- and extracapillary proliferation. These findings suggest that the initial clinical presentation of the illness allows for the prediction of the future.

Presentación clínica del absceso perinéfrico, de 1980 a 1985 / Clinical presentation of perinephric abscess from 1980 to 1985

Durante el periodo comprendido entre 1980 a 1985 se diagnosticaron 10 abscesos perinéfrico en siete mujeres y tres varones. La edad promedio fue de 43.1 años. La evolución promedio de 174.7 días. Los síntomas prominentes fueron dolor (costovertebral/abdominal), calosfríos repetidos y fiebre. A la exploración física se encontró una tumoración en nueve de los diez casos. La hemoglobina promedio fue de 9.94 con un recuento total de leucocitos de 16,360. Todos los pacientes tenían alteraciones en la urografía excretora sugestivas de absceso perinéfrico. Las enterobacterias gramnegativas sensibles predominaron (siete de 10) como los agentes etiogénicos. Esta entidad se debe sospechar en mujeres/varones mayores de 40 años, con diabetes sacarina no insulinodependiente, anémicos, con duración de signos y síntomas de más de una semana, a los que se les practicó una urografía excretora

Nefritis postestreptocócica en pediatría: experiencia en 45 casos / Post-streptococcal nephritis in pediatrics: experience in 45 cases

Se analizan 45 casos de nefritis postestreptocócica observados durante 1983, destacando: a) un marcado incremento de casos concordante con el mayor número de consultas por escabiosis infectada; b) la evolución favorable del cuadro con menor permanencia hospitalaria, y c) la utilidad de la determinación de C3. Se resaltan los aspectos clínicos importantes

Nephritis in systemic lupus erythematosus in children.

Twenty-five patients, aged six to 18 years, with lupus nephritis, followed for two to 103 months (median 22 months), have been classified according to renal histology into groups with diffuse proliferative glomerulonephritis (18), focal proliferative nephritis (2), and membranous nephropathy (5). Correlations have been made among specific histologic groups, clinical findings, renal function studies, urinary findings, and response to therapy. In sequential renal biopsies, lesions progressed in most patients with diffuse proliferative and membranous changes; however, chronic renal failure occurred in only one patient and the five-year survival rate (60.9%) is better than previously reported in pediatric patients.