Abstract: SA-PO1098
Non-Lupus Immune-Complex Glomerulonephropathy (ICGN) After Kidney Transplantation: A Report of 9 Cases
Session Information
- Pathology and Lab Medicine: Clinical
October 27, 2018 | Location: Exhibit Hall, San Diego Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Pathology and Lab Medicine
- 1502 Pathology and Lab Medicine: Clinical
Authors
- Chin, Kuo-Kai, Stanford University School of Medicine, Stanford, California, United States
- O'Shaughnessy, Michelle M., Stanford University School of Medicine, Stanford, California, United States
- Grimm, Paul C., Stanford University School of Medicine, Stanford, California, United States
- Troxell, Megan L., Stanford University School of Medicine, Stanford, California, United States
- Cheng, Xingxing S., Stanford University School of Medicine, Stanford, California, United States
Background
ICGN with positive immunofluorescence (IF) for IgG, IgA, IgG, C3, and C1q has been termed “non-lupus full-house nephropathy (FHN)” in the absence of clinical or serologic features of systemic lupus erythematosus (SLE). We describe characteristics and outcomes of ICGN with IgG, C3, and C1q +/- IgM/IgA deposits in the kidney allograft.
Methods
We retrospectively reviewed a single-institution pathology database from 2007-2017 to identify cases of allograft ICGN with IgG, C3 and C1q in the absence of SLE. Clinicopathologic and outcome data were derived from patient records.
Results
Of 9 patients, most were non-white (89%), male (89%), and pediatric-adolescent (66%). None had ICGN as cause of ESRD. Median time from transplant to ICGN was 1.6 (range 0.5-5.2) years and 5/9 (56%) were identified on surveillance biopsy. Two of 7 patients had a positive donor-specific antibodies. By light microscopy, 7 cases had mesangial hypercellularity, and two had FSGS. At last follow-up (median 3.9 years, range 0.5-13.8), 7 of 9 patients (78%) had a stable serum creatinine with minimal proteinuria in 6. The remaining 2 patients had persistent allograft dysfunction. Four patients had subsequent biopsies with immunofluorescence: 3 (75%) showed persistent ICGN while 1 (25%) had resolution of immune complexes.
Conclusion
Post-transplant ICGN with IgG, C3 and C1q may be clinically silent and/or resolve spontaneously and in most cases appears to follow an indolent clinical course. Whether immune complex formation results from an auto- or allo-immune response remains to be elucidated.
| Pt # | Age | Sex | Race | Native Disease (Bx, Y/N) | Years Post-Tx | Indication | Light Microscopy Findings | Therapy Change | Years Post-Bx | Follow-Up |
| 1 | 73 | M | Other | Diabetic (N) | 5.2 | sCr 1.5, uPCR 2.9 | FSGS, MICD-HY | None | 3.9 | Death, sCr 0.8 |
| 2 | 20 | M | Pacific Islander | FSGS (N) | 1.6 | Surv | MICD-HY | None | 2.5 | sCr 1.6 |
| 3 | 19 | M | White | Alport (N) | 1.3 | Surv | MICD-HY | Steroid Pulse, increase MMF | 9.3 | sCr 0.7, uPCR 2.0 |
| 4 | 16 | M | Pacific Islander | Unknown (N) | 2.9 | sCr 1.5, uPCR 1.1 | MICD-HY | Increase MMF | 13.8 | sCr 3.1, uPCR 7.0 |
| 5 | 12 | M | Unknown | Methylmalonic Acidemia (N) | 0.5 | Surv | MICD-HY | Add Steroid | 0.9 | sCr 0.6 |
| 6 | 4 | M | Black | Denys-Drash (N) | 1.1 | Surv | MICD-HY | None | 5.0 | sCr 0.8 |
| 7 | 19 | F | Other | FSGS (Y) | 2.3 | sCr 1.3 | MICD | None | 4.1 | sCr 0.9, uPCR 0.12 |
| 8 | 32 | M | Asian | IgA (Y) | 1.0 | Surv | MICD-HY | None | 0.6 | sCr 1.2, uPCR 0.29 |
| 9 | 60 | M | Unknown | Hypertension (N) | 1.6 | sCr 1.9, uPCR 1.1 | FSGS, MICD | Steroid Pulse | 0.5 | sCr 1.9 |
Tx = Transplant; Bx = biopsy; FSGS = Focal Segmental Glomerulosclerosis; HY = hypercellularity; sCr = serum creatinine, mg/dL; uPCR = urine protein-creatinine ratio, g/g; surv = surveillance; MICD = mesangial immune complex deposition; MMF = mycophenolate mofetil