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Nephrology
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Case Report: Collagenofibrotic Glomerulopathy After Transplant

Posted on March 17, 2020

In this case report, a 66-year-old man with end-stage renal disease related to antineutrophil cytoplasmic autoantibody (ANCA)-negative pauci-immune crescentic glomerulonephritis, coronary artery disease, atrial fibrillation, and hypertension, is discussed. In December 2016, the patient underwent a living unrelated renal transplant via paired exchange with an anti-rejection drug regimen of tacrolimus, everolimus, and prednisone.

Post-transplant creatinine nadir was 1.5 mg/dl -2.0 mg/dl but rose to 3.0 mg/dl – 3.8 mg/dl before his evaluation. The patient had a negative donor-specific antibodies (DSA), low positive BK blood titers (peak of 244,000 copies/mL down to 838 copies/mL), and proteinuria of 500 mg/g – 1000 mg/g.

Thirty-percent interstitial fibrosis and tubular atrophy was demonstrated after a renal transplant biopsy. Arteriolar hyalinosis secondary to calcineurin inhibitor toxicity with no evidence of transplant rejection was also noted.

Glomeruli showed focal accumulation of PAS pale material in the capillary lumina and in the mesangium. Immunofluorescence was negative. On electron microscopy, there were subendothelial and mesangial deposits of curvilinear collagen fibrils compatible with collagenofibrotic glomerulopathy.

There was no evidence of similar deposits on the native renal biopsy and the donor’s records showed serum creatinine of 0.93 mg/dl with no significant proteinuria. The patient was transitioned from tacrolimus to belatacept with improvement in his serum creatinine to 2.34 mg/dl.

The authors concluded that, “collangenofibrotic glomerulopathy is an extremely rare disease and in this case, it is unclear whether it was donor derived or developed de novo after renal transplant. Renal biopsy of the donor would be the definitive diagnostic test but was not indicated with normal donor kidney function.”

Reference

Gilligan S, Raghavan S, Revelo Penafiel MP, et al. Collagenofibrotic glomerulopathy in a renal transplant patient. J Am Soc Nephrol 30. 2019; Kidney Week 2019 Supplement.

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