KV Kanodia¹, AV Vanikar¹, RD Patel¹, PR Shah², M Nagpal², A Firoz², J Falodia², P Kasat², HL Trivedi^2
1 Department of Pathology, Laboratory Medicine, Transfusion Services and Immunohematology, Dr. H L Trivedi Institute of Transplantation Sciences (ITS)-Smt Gulabben Rasiklal Doshi and Smt Kamlaben Mafatlal Mehta Institute of Kidney Diseases and Research Centre (IKDRC), Civil Hospital Campus, Asarwa, Ahmedabad, Gujarat, India
² Department of Nephrology and Clinical Transplantation, Dr. H L Trivedi Institute of Transplantation Sciences (ITS)-Smt Gulabben Rasiklal Doshi and Smt Kamlaben Mafatlal Mehta Institute of Kidney Diseases and Research Centre (IKDRC), Civil Hospital Campus, Asarwa, Ahmedabad, Gujarat, India

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Abstract

Collapsing glomerulopathy (CG), characterized histologically by segmental/global glomerular capillary collapse, podocyte hypertrophy and hypercellularity and tubulo-interstitial injury; is characterized clinically by massive proteinuria and rapid progressive renal failure. CG is known to recur in renal allograft and rarely de novo. We report de novo CG 3 years post­transplant in a patient who received renal allograft from haplo-identical type donor.

Introduction

is regarded as a distinctive variant of focal segmental glomerulosclerosis (FSGS). ^[1] CG is known to recur in renal allograft and rarely de novo. ^[2]

A morphologically similar lesion has been described in human immuno deficiency virus (HIV) associated nephrotic syndrome. ^[3] The pathogenesis of CG is unknown.

We report de novo CG 3 years post-transplant in a patient who received renal allograft from haploidentical donor.

Case Report

A 28-year-old man underwent renal trans­plantation for hypertension induced end-stage renal disease in our institute on 26 ^th February 2003, with haploidentical kidney from his mother. He maintained creatinine around 1.6 mg/dL till April 2006 when a gradual rise in s. creatinine to 3.12 mg/dL was noticed over 4 months. He was maintained on standard triple drug immunosuppression of prednisolone 10 mg/day, azathiporine 100 mg/day, and cyclos­porine 2 mg/kg/day.

The laboratory investigations at time of biop­sy revealed urine albumin: 100 mg/day, 24­hour urinary protein: 3.27 gm, urine microscopy: 4–5 pus cells and 5-6 RBCs/ high power field, hemoglobin: 14.2 gm/dL, total WBC count: 8,200/mm ³ , and blood urea- 121 mg/dL. Hepa­titis HBs Ag was positive, and HIV, Hepatitis C Virus antibodies were non-reactive.

Renal biopsy adequate revealed 9 glomeruli, two medium caliber arteries, few small caliber arteries, and surrounding tubules. There was visceral cell hyperplasia with collapsed capil­lary tufts in 40 % of glomeruli and microcystic changes in tubules, [Figure 1],[Figure 2]. The remai­ning glomeruli revealed uniform mild mesan­gial matrix accentuation with segmental hyper­cellularity. Capillary lumina were fairly open and lined by mildly thickened membranes. Bowman capsule was thickened and revealed periglomerular fibrosis around one glomerulus. Tubules were moderately degenerated. Inters­titium was moderately prominent for focal fibrosis and mononuclear cellular infiltration (in 30 % of parenchyma). Small caliber arteries revealed segmental subintimal hyalinosis. No endothelialitis was observed. Immunofluore­scence studies were negative for all immune deposits including C4d. Therefore, the patient was diag-nosed as a case of de novo CG, and currently is on the waiting list for deceased donor transplantation program.

Discussion

Our patient with de novo CG presented at 41 months post-transplant with marked proteinu­ria and rapidly progressive allograft impair­ment. Hyaline arteriolosclerosis and interstitial fibrosis could be attributed to chronic cyclos­porine toxicity. Arterial and arteriolar narrow­wing besides glomerular collapse may be attri­buted to ischemia with cyclosporine toxicity. Meehan et al reported five HIV negative patients with de novo CG at 6–25 months post­transplant. ^[4] Immunofluorescence study in these cases revealed segmental mesangial deposits of IgM and C3. Electron microscopy illus­trated podocyte microvillous changes and seg­mental effacement of foot processes. However, we did not observe any immune deposits.

We conclude that De novo CG can occur rarely in a renal allograft with presentation of rapidly progressive graft failure. The patho­genesis of CG is not known. This report may encourage researchers for finding out its cause in order to improve graft survival.

 

References

1.	Detwiler RK, Falk R, Hogan S, Jennette JC. Collapsing glomerulopathy: A clinically and pathologically distinct variant of focal seg­mental glomerulosclerosis. Kidney Int 1994; 45:1416-24.
2.	Detwiler RK, Falk R, Hogan S, Jennette JC. Collapsing glomerulopathy in renal transplant patients: Recurrence and de novo occurrence. JAm Soc Nephrol 1996;7:1331A.
3.	D'Agati VV, Suh JI, Carbon L, Cheng JT, Appel G. Pathology of HIV associated nephro­pathy: a detailed morphologic and comparative study. Kidney Int 1989;35(6):1358-70.
4.	Meehan SM, Pascual M, William W, Tolkoff, Rubin N. De novo collapsing glomerulopathy in renal allograft. Transplantation 1998;65(9): 1192-7.

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Correspondence Address:
K V Kanodia
Department of Pathology, Laboratory Medicine, Transfusion Services and Immunohematology, Dr. H L Trivedi Institute of Transplantation Sciences (ITS)-Smt Gulabben Rasiklal Doshi and Smt Kamlaben Mafatlal Mehta Institute of Kidney Diseases and Research Centre (IKDRC), Civil Hospital Campus, Asarwa, Ahmedabad-380016, Gujarat
India

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PMID: 18711298

Figures

  [Figure 1], [Figure 2]