Subject(s)
Glomerulonephritis/immunology , Glomerulonephritis/pathology , Immunoglobulin M , Female , Humans , MaleABSTRACT
INTRODUCCIÓN: La nefropatía IgM (NIgM) es una glomerulonefritis caracterizada por depósitos mesangiales difusos de inmunoglobulina M (IgM), que suele manifestarse con proteinuria en rango nefrótico y que, según algunos trabajos previos, se presenta más frecuentemente en pacientes que son resistentes o dependientes del tratamiento con esteroides. OBJETIVO: Realizar una descripción clínica, histológica e inmunopatológica, y evaluar la respuesta al tratamiento esteroideo de los pacientes pediátricos con diagnóstico de síndrome nefrótico y depósitos mesangiales y difusos de IgM. MÉTODOS: Estudio descriptivo, retrospectivo, realizado en dos centros hospitalarios, donde se analizaron los registros clínicos de pacientes pediátricos con diagnóstico de NIgM y se revaluaron los cortes histológicos. RESULTADOS: Trece niños fueron incluidos en el presente estudio. La NIgM correspondió al 5,17 % de todas las biopsias renales pediátricas. La edad de los pacientes estuvo entre 1 y 12 años (mediana: 2 años); el 46,7 % fueron mujeres. El hallazgo morfológico más frecuente fue hipercelularidad mesangial difusa (46,1 %), seguido de glomeruloesclerosis focal y segmentaria (30,8 %) y cambios glomerulares mínimos (23,1 %). Todos los pacientes recibieron esteroides, en 4 de ellos (30,7 %) como el único medicamento inmunosupresor; 3 (23,1 %) recibieron además ciclofosfamida, 5 (38,4 %) micofenolato y 1 (7,7 %) ciclosporina. Siete pacientes (53,8 %) presentaron recaídas frecuentes, 5 (38,5 %) fueron corticorresistentes y 1 (7,7 %) corticodependiente. Dos pacientes (15,38 %) presentaron alteración crónica de la función renal. CONCLUSIÓN: La presencia de IgM mesangial difusa en pacientes pediátricos con síndrome nefrótico no es un hallazgo muy infrecuente, su presentación clínica se ha asociado con menor respuesta a esteroides. Sin embargo, su pronóstico a largo plazo aún no se conoce
INTRODUCTION: IgM nephropathy (IgMN) is a glomerulonephritis characterized by diffuse mesangial deposits of immunoglobulin M (IgM), which usually presents with nephrotic range proteinuria and, according to some previous studies, occurs most often in patients who are resistant or dependent of steroid treatment. AIM: To perform a clinical, histological and inmunopathological description, and assess response to steroid treatment of pediatric patients diagnosed with nephrotic syndrome and diffuse mesangial deposits of IgM. METHODS: This is a descriptive, retrospective study done in two hospitals. Clinical data were taken from medical records. All the slides with the histologic sections were re-evaluated. RESULTS: thirteen children were included in this study. IgMN corresponded to 5.17% of all pediatric renal biopsies. The age of patients ranged from 1 year to 12 years (median: 2 years), 46,15% were women. The most common morphological finding was diffuse mesangial hypercellularity (46,1%), followed by focal segmental glomerulosclerosis (30,8%) and minimal glomerular changes (23,1%). All patients received steroids, in 4 cases (30,7%) as the only immunosuppressant medication, 3 (23,1%) also received cyclophosphamide, 5 (38,4%) mycophenolate, and 1 (7.7%) cyclosporine. Seven patients (53,8%) had frequent relapses, 5 (38.5%) were cortico-resistant, and 1 (7.7%) corticodependent. Two patients (15,38%) had chronic impairment of renal function. CONCLUSION: The presence of diffuse mesangial IgM in pediatric patients with nephrotic syndrome is not a too uncommon finding, the clinical presentation has been associated with lower response to steroids, but their longterm prognosis is still unknown
Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Glomerulonephritis/diagnosis , Nephrosis, Lipoid/diagnosis , Glomerulosclerosis, Focal Segmental/diagnosis , Nephrotic Syndrome/diagnosis , Autoimmune Diseases , BiopsyABSTRACT
INTRODUCTION: IgM nephropathy (IgMN) is a glomerulonephritis characterised by diffuse mesangial immunoglobulin M (IgM) deposits. It usually presents with nephrotic range proteinuria and, according to some previous work, it occurs most often in patients who are resistant to or dependent on steroid treatment. OBJECTIVE: To perform a clinical, histological and immunopathological description and assess the response to steroid treatment of paediatric patients diagnosed with nephrotic syndrome and diffuse mesangial IgM deposits. METHOD: This is a descriptive, retrospective study carried out in two hospitals, where the clinical records of paediatric patients with IgMN were analysed and the histological sections were re-assessed. RESULTS: thirteen children were included in this study. IgMN corresponded to 5.17% of all paediatric renal biopsies. The age of patients ranged from 1 year to 12 years (median: 2 years), 46.7% were women. The most common morphological finding was diffuse mesangial hypercellularity (46.1%), followed by focal segmental glomerulosclerosis (30.8%) and minimal glomerular changes (23.1%). All patients received steroids; in 4 cases (30.7%) as the only immunosuppressant medication, 3 (23.1%) also received cyclophosphamide, 5 (38.4%) mycophenolate, and 1 (7.7%) cyclosporine. Seven patients (53.8%) had frequent relapses, 5 (38.5%) were cortico-resistant and 1 (7.7%) cortico-dependent. Two patients (15.38%) had chronic impairment of renal function. CONCLUSION: The presence of diffuse mesangial IgM in paediatric patients with nephrotic syndrome is not a very uncommon finding; its clinical presentation has been associated with lower response to steroids. However, the long-term prognosis of these patients is still unknown.
