ABSTRACT
The association between nephrotic syndrome and juvenile idiopathic arthritis have rarely been described in pediatric patients. We report a child with steroid-responsive nephrotic syndrome, with frequent relapses, who presented with a new relapse of nephrotic syndrome associated with arthritis and uveitis at 21 months in remission after treatment with chlorambucil. Juvenile idiopathic arthritis was diagnosed and kidney biopsy examination showed mesangial glomerulonephritis with immunoglobulin M deposits. To our knowledge, only 2 cases of nephrotic syndrome preceding juvenile idiopathic arthritis have been reported, one without histopathology assessment and the other with minimal change disease. Although mesangial glomerulonephritis with nephrotic syndrome and juvenile idiopathic arthritis could have been coincidental, the immune pathogenic mechanism accepted for both diseases suggests they could be related.
Subject(s)
Arthritis, Juvenile/immunology , Glomerulonephritis/immunology , Immunoglobulin M/analysis , Mesangial Cells/immunology , Nephrotic Syndrome/immunology , Anti-Inflammatory Agents/therapeutic use , Arthritis, Juvenile/diagnosis , Arthritis, Juvenile/drug therapy , Biopsy , Child, Preschool , Chlorambucil/therapeutic use , Glomerulonephritis/complications , Glomerulonephritis/diagnosis , Glomerulonephritis/drug therapy , Humans , Male , Mesangial Cells/pathology , Nephrotic Syndrome/diagnosis , Nephrotic Syndrome/drug therapy , Steroids/therapeutic use , Treatment OutcomeABSTRACT
El xantogranuloma juvenil es un tumor benigno secundario a una proliferación de células histiocíticas que se presenta en lactantes y niños; su aparición en adultos es ocasional. Se inicia en forma repentina por la aparición de lesiones cutáneas papulonodulares rojizo anaranjadas, redondeadas, de 2 a 6 mm, que se localizan preferentemente en cara y parte superior del cuerpo. Se resuelve en forma espontánea en un período variable. Las lesiones suelen estar limitadas a la piel pero pueden tener otra localización. La ocular es una de las que puede presentar severas complicaciones que incluyen glaucoma, hipema, proptosis y amaurosis como en la paciente de 4 meses que presentamos.
Juvenile xantogranuloma (JXG) is a benign tumor due to the prolifera-tion of histiocytic cells, which appears in infants and children and occasionanlly in adults. It has a sudden onset which consists of erythematous or yellowish, papulonodular, slightly raised lesions, with a diameter varying from 2 to 6 mm. It is usually located on the face and upper part of the body, and has a spontaneous remission in a variable period of time. Although lesions are usually limited to the skin, other organs may be involved. Ocular involvement may lead to severe complications including glaucoma, hyphema, proptosis and blindness, as the four months-old patient we report.
