Goodpastures Syndrome With Ambiguous Serology: A Case Report

Abstract

Pulmonary-renal syndrome is a rare clinical syndrome defined by a combination of diffuse alveolar hemorrhage and rapidly progressive crescentic glomerulonephritis. It is not a single entity, but is caused by a wide variety of diseases including Goodpastures syndrome associated with autoantibodies against glomerular and alveolar basement membrane (anti-glomerular basement membrane (anti-GBM)). Anti-GBM disease is an autoimmune disorder, estimated to occur in less than one case per million population. A 59-year-old previously healthy man presented with acute kidney injury (AKI, stage 3, KDIGO) with signs of nephritic syndrome, progressive oliguria, dyspnea with mild hemoptysis, cruses edema and elevated blood pressure. Serologic results were all negative except very low titer of anti-proteinase 3 antibodies. Chest radiograph revealed diffuse pulmonary infiltrations. Renal biopsy demonstrated necrotizing crescentic glomerulonephritis with linear pattern for immunoglobulin G along the entire GBM in immunofluorescent staining. Based on whole clinical picture, the diagnosis of Goodpastures syndrome was done and the intensive treatment, including plasmapheresis, cyclophosphamide and prednisone, along with hemodialysis was provided. Improvement of patients general condition was achieved. Despite recovery of diuresis, attempt to discontinue hemodialysis was found unsuccessful. The case proves the importance of diagnostic vigilance in patients presenting with AKI. Although supportive, in many cases, serology tests can be negative or ambiguous and should not prevent profound diagnostic process including kidney biopsy. Late diagnosis in the course of anti-GBM disease, in spite of proper treatment, correlates with less favorable kidney outcomes.




World J Nephrol Urol. 2017;6(1-2):10-13
doi: https://doi.org/10.14740/wjnu301w