Abstract
We report a case of a 10-year-old boy with Goodpasture syndrome double-seropositive for anti-glomerular basement membrane (GBM) antibodies and anti-neutrophil cytoplasmic antibodies (ANCA). He was admitted to a hospital with 2 weeks of fatigue and loss of appetite. Blood tests revealed serum creatinine of 8.19 mg/dl and positive results for both serum anti-GBM antibodies and ANCA. Chest computed tomography (CT) showed diffuse ground-glass opacities (GGO) suggesting pulmonary hemorrhage. He was referred to our hospital and treated with six rounds of plasma exchange (PE) and three courses of methylprednisolone pulse therapy. Both serum anti-GBM antibodies and ANCA were undetectable, and the follow-up chest CT demonstrated the disappearance of GGO, but his renal function showed no improvement. Renal biopsy disclosed 98% sclerotic glomeruli and linear IgG depositions along the GBM. Nocturnal peritoneal dialysis was initiated, and prednisolone and mycophenolate mofetil were administered as maintenance therapy. Remissions occurred for 1 year after the initiation of treatment. Although he showed an end-stage kidney disease stage at admission, he was aggressively treated with PE and immunosuppressive therapy due to pulmonary hemorrhage. Owing to a relatively high risk of relapse, long-term immunosuppressive maintenance therapy should be considered for anti-GBM disease double-seropositive for anti-GBM antibodies and ANCA.
