ESPN 51th Annual Meeting

ESPN 2018


 
An unusual ‘‘crescentic glomerulonephritis’’ in a kidney-transplanted child
VASILIKI KARAVA 1 ARGYROULA ZAMPETOGLOU 1 SMARAGDI MARINAKI 2 MARIA BITSORI 3 HARA GIAKOPOULOU 4 CONSTANTINOS J. STEFANIDIS 1 ANDROMACH MITSIONI 1

1- PEDIATRIC NEPHROLOGY DEPARTMENT, "P. AND A. KYRIAKOU" CHILDRENS HOSPITAL, ATHENS, GREECE
2- NEPHROLOGY DEPARTMENT AND RENAL TRANSPLANTATION UNIT, LAIKO HOSPITAL, NATIONAL AND KAPODISTRIAN UNIVERSITY OF ATHENS, GREECE
3- DEPARTMENT OF PAEDIATRICS, HERAKLION UNIVERSITY HOSPITAL, HERAKLION, GREECE
4- 1ST DEPARTMENT OF PATHOLOGY, SCHOOL OF MEDICINE, NATIONAL & KAPODISTRIAN UNIVERSITY OF ATHENS, GREECE
 
Introduction:

 We report a case of anti-HLA antibody mediated rejection (ABMR) presented as a crescentic glomerulonephritis in a kidney transplanted child.

Material and methods:

A 10-year old boy with Fin major Finnish type nephrotic syndrome, who had received a deceased-donor kidney transplant since 5 years, presented acute kidney injury associated to nephrotic proteinuria. His immunosuppressive treatment included tacrolimus, mycophenolate mofetil and prednisone at low dose. Renal biopsy was performed which contained 12 glomeruli in total; 6 with global sclerosis and 6 with remarkable circumferential cellular crescents. There were no signs of cellular rejection whereas glomerulitis (g=3) and peritubular capillaritis (pct=2), as well as acute tubular injury were noticed. Moreover, glomerular basement membrane double contours were observed. C4d staining of peritubular capillaries was positive. There was no positivity for immunoglobulins, complement factors and light chains in immunofluorescence. 

 

Results:

Pauci-immune glomerulonephritis was excluded since exploration for serum anti-glomerular basement membrane autoantibodies (anti-GBM) and anti-neutrophil cytoplasmatic autoantibodies (anti-ANCA) was negative. However, levels of HLA class II donor specific antibodies were remarkably high with a mean fluorescence intensity (MFI) of 23000 UI/ml for HLA DQ antibodies. These findings confirmed the diagnosis of acute associated to chronic ABMR. The patient was submitted to ten sessions of plasmapharesis replacement and received 3 pulses of methylprednisolone, one dose of Rituximab and two infusions of high dose human immunoglobulin. Nevertheless, the patient developed end stage renal disease and renal replacement therapy started 4 months later.

Conclusions:

Glomerular crescents may represent a finding of AMBR, secondary to severe antibody interaction with the vascular endothelium. Peritubular C4d is important for the diagnosis of AMBR, when de novo glomerulonephritis is suspected. HLA class II mismatching, especially DQ, is a major determinant of DSA and must prompt considerations of optimizing matching before kidney transplantation.