Undiagnosed IgA Nephropathy as a Cause of End Stage Renal Disease Revealed after Early Recurrence in a Kidney Graft

Case Report

Austin J Nephrol Hypertens. 2015; 2(4): 1044.

Undiagnosed IgA Nephropathy as a Cause of End Stage Renal Disease Revealed after Early Recurrence in a Kidney Graft

Trimarchi H¹*, Rengel T¹, Andrews J¹, Paulero M¹, Forrester M¹, Lombi F¹, Pomeranz V¹, Iriarte R¹ and Iotti A²

¹Department of Nephrology and Pathology Services, Hospital Británico de Buenos Aires, Argentina

²Department of Power Plant Technology, University of Pheonix, Argentina

*Corresponding author: Hernan Trimarchi, Department of Nephrology and Pathology Services, Hospital Británico de Buenos Aires, Perdriel 74 (1280), Buenos Aires, Argentina

Received: August 24, 2015; Accepted: October 30, 2015; Published: November 01, 2015


An 18 year-old male was started on chronic hemodialysis due to glomerulonephritis. Despite any previous clinical symptom related to glomerular involvement, and foamy urines were assumed as normal. In a routine medical examination, he was found to be anemic. Advanced kidney failure was diagnosed and heavy proteinuria and micro hematuria were reported. A kidney biopsy revealed global glomerulosclerosis with copious non-specific inmunoglobulina mesangial deposition. Six months there after he received a kidney graft from his mother. Due to an increase in serum creatinine without hematuria and mild proteinuria 7 days after transplantation, a kidney graft biopsy revealed acute tubular necrosis and normal glomeruli. Kidney function improved. Three months after transplantation, when immunosuppression consisted on maintenance lowdose meprednisone, mycophenolate and tacrolimus, creatinine levels creped together with proteinuria and dysmorphic hematuria. A second kidney biopsy revealed IgA nephropathy, which was interpreted as the possible primary undiagnosed cause of end-stage kidney disease.

Keywords: IgA nephropathy; Proteinuria; Kidney transplantation; Hematuria


IgA Nephropathy (IgAN) is the most common primary glomerulonephritis, characterized by the presence of micro or macrohematuria, proteinuria and diffuse mesangial depositions of IgA in the kidney biopsy. Between 15 to 40% of patients develop chronic kidney disease after 20 years of diagnosis, and 20% show of cases display significant decreases in renal function [1]. IgAN affects both genders, being more common in men than in women. It is classically diagnosed between the second and forth decade of life. It rarely affects African Americans, being more common in Asians and Caucasians. In frequency, it is the second cause encountered in kidney biopsies in children, while it is the fourth in patients over 65 years, after vasculitis, membranous nephropathy and primary amyloidosis [1]. KDIGO 2012 guidelines for the treatment of glomerulonephritis recommend for IgAN in a first step approach the use of drugs that block the renin angiotensin system, as angiotensin-converting enzyme inhibitors, angiotensin II receptor antagonist, or both (dual blockade), due to their anti-proteinuric effects and protective actions on the podocytes and the slit diaphragm [2]. However, IgAN recurs frequently in the kidney transplants, and can compromise the graft survival in the long term [3-6].

Case Presentation

An eighteen year-old male patient was referred for nephrology assessment. He had a history of recently diagnosed severe proteinuria (7g/day), dysmorphic hematuria and a creatinine clearance of 18 mL/min. He was also found to be anemic and hypertensive. A kidney biopsy was performed, which reported diffuse global glomerulosclerosis with tubular foamy cells, histological features of chronic interstitial nephritis, and severe interstitial fibrosis and tubular atrophy. Immunofluorescence was positive for total Ig, and dominant mesangial deposits of IgM and of IgG and IgA in a lower extent. The patient was started on hemodialysis and a living-related kidney transplant assessment was initiated. Six months thereafter, he received a kidney from his mother. Induction immunosuppression consisted on thymoglobulin, and maintenance therapy consisted on sodium mycophenolate, meprednisone and tacrolimus. Seven days post-transplant a kidney graft biopsy was performed due to serum creatinine elevation to 2.3mg/dL. Histology findings consisted on mild acute tubular necrosis, C4d negative staining, and without glomerular immune deposits in the immunofluorescence. Anti-HLA antibodies were negative. A month later serum creatinine decreased to 1.6 mg/dL, remaining at this level. Three months post-transplant serum creatinine rose to 2.26 mg/dL, proteinuria 1.4 g/day and the urinary smear showed 15 red blood cells per high power field, being 70% dysmorphic plus acanthocytes. Tacrolimus through levels were within normal limits. BK virus was negative. A new kidney biopsy was performed, which showed a slight increase in the glomerular matrix and mesangial cells, and mild (5%) tubular atrophy and interstitial fibrosis (Oxford score MoEoSoTo). Immunofluorescence disclosed predominant IgA deposits. Electron microscopy revealed mesangial electron dense deposits. Patient was rechallenged on 1mg/ kg of meprednisone for eight weeks, mycophenolate was halved and tacrolimus was continued to achieve trough levels between 5-10 ng/ mL. Enalapril 10 mg/day was added. Six months post-transplant hematuria disappeared and proteinuria dropped to 0.7 g/day. Serum creatinine was 2.3 mg/dL while the estimated glomerular filtration rate was 50 mL/min.