De novo lupus-like glomerulonephritis after pediatric non-kidney organ transplantation

<jats:title>Abstract</jats:title><jats:sec> <jats:title>Background</jats:title> <jats:p>We propose a novel clinically significant finding, de novo lupus-like glomerulonephritis (DNLLGN), in patients with autoantibodies and kidney abnormalities in pediatric liver transplant (LT) and intestinal inclusive transplants (ITx).</jats:p> </jats:sec><jats:sec> <jats:title>Methods</jats:title> <jats:p>We describe the clinical, serologic, and histopathologic presentation and kidney outcomes in eight patients from our center found to have DNLLGN on kidney biopsy.</jats:p> </jats:sec><jats:sec> <jats:title>Results</jats:title> <jats:p>Pediatric recipients of non-kidney solid organ transplants developed an unusual de novo immune complex glomerulonephritis with morphologic similarity to lupus nephritis. Six had isolated LT (0.9% of all pediatric LT at our center) and two had ITx (2.1% of all ITx). Five (63%) presented with nephrotic syndrome. Five patients had autoantibodies. Patients underwent kidney biopsy at a mean of 11.5 years in LT and 2.8 years in ITx after the index transplant. Biopsies demonstrated changes similar to focal or diffuse active lupus. Follow-up eGFR at a mean of 6 years after biopsy showed a mean decrease of 30 ml/min/1.73 m<jats:sup>2</jats:sup> in all patients (p = 0.11).</jats:p> </jats:sec><jats:sec> <jats:title>Conclusions</jats:title> <jats:p>DNLLGN has not been previously recognized in this clinical setting, yet 8 kidney biopsies from pediatric recipients of LT and ITx at our center in 25 years demonstrated this finding. DNLLGN appears to be an under-reported phenomenon of clinical significance.</jats:p> </jats:sec><jats:sec> <jats:title>Graphical abstract</jats:title> </jats:sec>