ABSTRACT
Goodpasture Syndrome is described as a single episode disease entity. It is diagnosed with the demonstration of antiglomerular basement (anti-GBM) antibodies in plasma or renal tissue. Although the recurrence of anti-GBM disease is rare, it has been reported in up to 3% of cases. Recurrence with negative anti-GBM antibodies in plasma is even less frequent We report a 63 years old male in whom anti-GBM disease recurred without detectable anti-GBM antibodies in plasma, despite having positive antibodies at the onset.
Subject(s)
Humans , Male , Middle Aged , Autoantibodies/analysis , Anti-Glomerular Basement Membrane Disease/pathology , Recurrence , Biopsy , Prednisone/therapeutic use , Trimethoprim, Sulfamethoxazole Drug Combination/therapeutic use , Fluorescent Antibody Technique , Anti-Glomerular Basement Membrane Disease/drug therapy , Anti-Glomerular Basement Membrane Disease/diagnostic imaging , Cyclophosphamide/therapeutic use , Kidney Diseases/pathology , Kidney Glomerulus/pathology , Anti-Bacterial Agents/therapeutic useABSTRACT
El síndrome de Goodpasture es más frecuente en varones, adultos jóvenes. Se presento una paciente de sexo femenino de doce años de edad con historia de palidez, astenia y adinamia, expectoración hemoptoica y dificultad respiratoria rápidamente progresiva, anemia microcítico y falla renal aguda que evolucionó con insuficiencia respiratoria grave e irreversible secundario a hemorragia pulmonar masiva. Los exámenes anatomopatológicos mostraron hemosiderosis pulmonar y glomerulonefritis crecéntica, compatibles con síndrome de Goodpasture
Subject(s)
Humans , Female , Anti-Glomerular Basement Membrane Disease/diagnosis , Anti-Bacterial Agents/therapeutic use , Cause of Death , Diagnosis, Differential , Glomerulonephritis , Hemosiderosis , Iron/therapeutic use , Anti-Glomerular Basement Membrane Disease/physiopathology , Anti-Glomerular Basement Membrane Disease/drug therapyABSTRACT
El síndrome Good Pasture es un cuadro poco frecuente, de patogenia inmuno-reactiva en el que existen autoanticuerpos antimembrana basal de glomérulos renales y de alvéolos pulmonares. Afecta de preferencia a hombres jóvenes y se maniiesta fundamentalmente por sintomatología respiratoria (hemoptisis y disnea); renal (proteinuria, hematuria y edemas) y anemia. El pronóstico es grave y el tratamiento (corticoides, inmunosupresores y plasmaféresis) es tanto más efectivo mientras más precozmente se instituya