Table 3.
Clinical features, according to immune-complex glomerulopathy not otherwise specified (ICG-NOS) subgroup
| Total (n = 28) | Full-house (+IgG/+IgM/+IgA/+C3/+C1q) (n = 3) |
Quasi-full-house (+IgG/+C3/+C1q) (n = 10) |
IgA-rich (IgA-codominant) (n = 3) |
C1q rich (−IgG/+C1q) (n = 6) |
C1q poor (+IgG/−C1q) (n = 6) |
P value | |
|---|---|---|---|---|---|---|---|
| Age at transplantation, median (range) | 17.5 (1–71) | 18 (1–68) | 17 (2–71) | 24 (17–31) | 19 (15–54) | 14 (8–26) | 0.53 |
| Adult at transplantation, n (%) | 14 (50) | 2 (67) | 5 (50) | 2 (67) | 3 (50) | 2 (33) | 0.86 |
| Male, n (%) | 17 (61) | 3 (100) | 7 (70) | 2 (67) | 3 (50) | 2 (33) | 0.34 |
| Race/ethnicity, n (%) | 0.48 | ||||||
| Caucasian | 8 (29) | 0 (0) | 2 (20) | 1 (3) | 2 (33) | 3 (50) | |
| Hispanic | 8 (29) | 2 (67) | 2 (20) | 0 (0) | 3 (50) | 1 (17) | |
| African American | 2 (7) | 0 (0) | 1 (10) | 0 (0) | 1 (17) | 0 (0) | |
| Asian/Pacific Islander | 6 (21) | 1 (33) | 2 (20) | 2 (67) | 0 (0) | 1 (17) | |
| Other | 4 (14) | 0 (0) | 3 (30) | 0 (0) | 0 (0) | 1 (17) | |
| Cause of ESRD, n (%) | 0.47 | ||||||
| Nonimmune ESRDa | 9 (32) | 1 (33) | 3 (30) | 0 (0) | 1 (17) | 4 (67) | |
| Systemic autoimmune disease | 2 (7) | 0 (0) | 0 (0) | 1 (33) | 0 (0) | 1 (17) | |
| Glomerulonephritisb | 7 (25) | 1 (33) | 2 (20) | 1 (33) | 2 (33) | 1 (17) | |
| Unknown/unbiopsied | 10 (36) | 1 (33) | 5 (50) | 1 (33) | 3 (50) | 0 (0) | |
| Time from transplant to biopsy, years, median (range) | 2.1 (0.2–11.1) | 4.3 (1.6–5.2) | 2.0 (0.5–4.3) | 3.5 (0.9–9.0) | 2.1 (0.2–5.9) | 1.1 (0.4–11.1) | 0.75 |
| Biopsy indication, n (%) | 0.47 | ||||||
| Increased sCr | 7 (25) | 1 (33) | 2 (20) | 1 (33) | 2 (33) | 1 (17) | |
| Proteinuria | 3 (11) | 1 (33) | 2 (20) | 0 (0) | 0 (0) | 1 (17) | |
| Increased sCr and proteinuria | 4 (14) | 0 (0) | 2 (20) | 0 (0) | 1 (17) | 0 (0) | |
| Positive DSA | 1 (4) | 0 (0) | 0 (0) | 1 (33) | 0 (0) | 0 (0) | |
| Rejection follow-up | 1 (4) | 0 (0) | 0 (0) | 0 (0) | 1 (17) | 0 (0) | |
| Protocol | 12 (43) | 1 (33) | 4 (40) | 1 (33) | 2 (33) | 4 (67) | |
| Laboratory features at time of biopsy | |||||||
| sCr (mg/dl), median (range) | 1.2 (0.4–7.0) | 1.1 (0.7–1.5) | 1.1 (0.4–2.0) | 1.2 (1.0–2.8) | 1.2 (0.9–3.5) | 0.9 (0.7–7.0) | 0.54 |
| Hematuria, n (%) | 8/22 (36) | 1/3 (33) | 1/7 (14) | 2/3 (67) | 1/4 (25) | 3/5 (60) | 0.39 |
| Proteinuria, n (%) | 11/22 (59) | 1/3 (33) | 3/7 (43) | 1/3 (33) | 1/4 (25) | 3/5 (60) | 0.77 |
| Positive DSA, n (%) | 12/19 (63) | 1/1 (100) | 3/7 (43) | 1/2 (50) | 4/4 (100) | 3/5 (60) | 0.34 |
| ANA+, n (%) | 3/12 (25) | 1/1 (100) | 1/5 (20) | 0/2 (0) | 0/3 (0) | 1/1 (100) | 0.53 |
| Low C3, n (%) | 3/12 (25) | 1/1 (100) | 2/8 (25) | 0/2 (0) | 0/1 (0) | 0/0 (n/a) | 0.04 |
| Low C4, n (%) | 2/12 (17) | 1/1 (100) | 1/8 (12.5) | 0/2 (0) | 0/1 (0) | 0/0 (n/a) | 0.11 |
| Known infection at time of Bx, n (%) | 6 (21) | 1/3 (33) | 2/10 (20) | 0/3 (0) | 0/6 (0) | 3/6 (50) | 0.23 |
| History of viral activation | 9 (32) | 2/3 (67) | 3/10 (30) | 0/3 (0) | 2/6 (33) | 2/6 (33) | 0.54 |
| Antibody-mediated rejection | 0.10 | ||||||
| Confirmed | 9 (32) | 1 (33) | 1 (10) | 1 (33) | 4 (67) | 2 (33) | |
| Suspicious | 3 (11) | 0 (0) | 0 (0) | 1 (33) | 0 (0) | 2 (33) | |
| T-cell–mediated rejection | 0.07 | ||||||
| Confirmed | 1 (4) | 1 (33) | 0 (0) | 0 (0) | 0 (0) | 0 (0) | |
| Borderline | 9 (32) | 0 (0) | 5 (50) | 1 (33) | 3 (50) | 0 (0) | |
| No rejection | 12 (43) | 2 (67) | 5 (50) | 1 (33) | 2 (33) | 2 (33) | 0.83 |
| Treatment, n (%) | |||||||
| Steroid pulse | 9 (32) | 1 (33) | 4 (40) | 2 (67) | 2 (33) | 0 (0) | 0.31 |
| I.v. IG | 6 (21) | 1 (33) | 1 (10) | 1 (33) | 3 (50) | 0 (0) | 0.21 |
| Increase immunosuppression | 8 (29) | 0 (0) | 1 (10) | 1 (33) | 5 (83) | 1 (17) | 0.02 |
| No change | 14 (50) | 2 (67) | 5 (50) | 1 (33) | 1 (17) | 5 (83) | 0.20 |
| Yr of follow-up post-Bx, median (range) | 2.3 (0.1–14.0) | 1.9 (0.3–3.9) | 2.5 (0.6–14.0) | 1.1 (1.1–3.4) | 3.6 (0.6–7.9) | 2.5 (0.1–3.3) | 0.47 |
| Graft failure, n (%) | 7 (25) | 0 (0) | 2 (20) | 2 (67) | 2 (33) | 2 (33) | 0.79 |
| Yr to graft failure post-Bx, median (range)c | 3.0 (0.1–14.0) | n/a | 8.8 (3.6–14.0) | 1.1 | 2.3 (0.6–3.9) | 1.5 (0.1–3.0) | 0.42 |
| Laboratory features at time of last follow-upd | |||||||
| sCr at last follow-up (mg/dl), median (range) | 1.0 (0.5–5.2) | 1.0 (0.5–1.5) | 0.85 (0.5–2.4) | 3.3 (1.4–5.2) | 1.8 (0.9–3.0) | 0.8 (0.7–1.1) | 0.69 |
| Hematuria, n (%) | 4/16 (25) | 0/2 (0) | 1/6 (17) | 1/2 (50) | 1/2 (50) | 1/4 (25) | 0.70 |
| Proteinuria, n (%) | 6/17 (35) | 0/2 (0) | 2/7 (28) | 2/2 (100) | 1/2 (50) | 1/4 (25) | 0.26 |
| Follow-up biopsy with IF | 8/28 (29) | 1/3 (33) | 5/10 (50) | 0/3 (0) | 0/6 (0) | 2/6 (33) | 0.51 |
| Follow-up immune deposition | 0.11 | ||||||
| Persistent | 2 (25) | 1 (100) | 0 (0) | 1 (50) | |||
| Improved | 6 (75) | 0 (0) | 5 (100) | 1 (50) | |||
| Death, n (%) | 1 (4) | 1 (33) | 0 (0) | 0 (0) | 0 (0) | 0 (0) | 0.07 |
ANA, antinuclear antibody; Bx, biopsy; DSA, donor-specific antibody; ESRD, end-stage renal disease; IF, immunofluorescence; sCr, serum creatinine.
For laboratory values at time of biopsy, the denominators (as shown in the first column) are frequently smaller than 28, as testing was not performed in all patients.
Includes presumed diabetic nephropathy, presumed hypertensive nephropathy, polycystic kidney disease, methylmalonic acidemia, reflux.
Of the 7 patients who had glomerulonephritis (GN) as a cause of ESRD, 5 had posttransplant GN that was phenotypically distinct from the original disease (focal and segmental glomerulosclerosis = 4, Churg-Strauss = 1). Two had potentially related disease: 1 patient with IgA nephropathy developed IgA-rich ICG-NOS; 1 patient with crescentic glomerulonephritis developed C1q+ ICG-NOS, but no evidence of crescents.
For patients who reached graft failure.
Excludes patents with graft failure.