Session Information
Session Type: Abstract Submissions (ACR)
Background/Purpose : LN is common in childhood-onset Systemic Lupus Erythematosus (cSLE). Kidney biopsies are impractical to assess the course of LN given their invasiveness and cost. Therefore, traditional laboratory measures [GFR, complement levels, anti-dsDNA antibodies, serum creatinine and urinary protein/creatinine ratio] are used and several clinical indices [Systemic Lupus International Collaborating Clinics Renal Activity Score (SLICC-RAS), renal domain score of the BILAG (BILAG-R) and SLEDAI (SLEDAI-R)] have been developed: The objective of this study was to validate these traditional non-invasive measures of LN activity in cSLE, using histological activity & severity of LN as criterion standard.
Methods: The traditional laboratory measures were measured in 83 children with LN at time of the kidney biopsy. The biopsy specimens were rated by a single nephropathologist for LN severity as per International Societies for Nephrology & Renal Pathology (ISN/RPS) class, the NIH glomerular activity Index (GLAI; range 0-24) and the tubulointerstitial activity index (TIAI, range 0-21). For the statistical analysis, LN severity is categorized as Class I/II vs. III/IV vs. V; GLAI score (≤10 vs. >10), or TIAI score (≤5 vs. >5) in fixed effect models and logistical models, respectively.
Results: Of the 83 kidney biopsies, 12%, 60% and 28%, of the patients had class I/II, III/IV and V, respectively. The median scores of the GLAI and TIAI are summarized (Table 1). SLEDAI-R and SLICC-RAS, but not BILAG-R was positively associated to the ISN/RPS classification (Table 2). In particular, higher SLEDAI-R and serum creatinine level and lower GFR level was found in patients with LN inflammatory activity (GLAI > 10 or TIAI >5). Similar patterns were also noticed in patients with ISN/RPR Class III/IV being compared against those with ISN/RPR Class V.
Conclusion: Of the currently used measures to assess LN in routine daily practice, the SLEDAI-R and the GFR appears to best reflect inflammatory LN activity in both the glomerulus and the interstitium of pediatric populations.
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Table1. Summary of GLAI, TIAI and ISNPR CLASS in cSLE Cohort |
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|
Score |
Stat Type |
Statistics |
|
GLAI score |
Median (range) |
6 (0, 22) |
|
Freq (%) of score>10 |
27/71 (38.0%) |
|
|
TIAI score |
Median (range) |
4 (0, 11) |
|
Freq (%) % of score>5 |
14/55 (25.5%) |
|
|
ISNPR CLASS |
I/II |
10 (12.05%) |
|
III/IV |
50 (60.24%) |
|
|
V |
23 (27.71%) |
|
|
Table2 Traditional LN measures validated by LN biopsy |
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|
Traditional LN measure |
ISNRP Class |
GLAI Score |
AITI Score |
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|
I/II |
III/IV |
V |
≤ 10 |
> 10 |
≤ 5 |
> 5 |
|
|
SLEDAI-R* |
6.80 (3.75, 9.85) |
10.20 (8.84, 11.56)# |
6.78 (4.77, 8.79) |
7.45 (6.06, 8.84) |
11.93 (10.15, 13.70)‡ |
8.20 (6.75, 9.64) |
11.43 (8.96, 13.90)‡ |
|
BILAG-R* |
10.10 (8.26, 11.94) |
11.20 (10.38, 12.02) |
9.86 (8.62, 11.11) |
10.37 (9.52, 11.23) |
11.56 (10.48, 12.63) |
10.93 (10.02, 11.83) |
10.86 (9.33, 12.38) |
|
SLICC-RAS* |
3.11 (0.45, 6.67) |
6.96 (5.42, 8.50) |
5.83 (3.32, 8.35) |
4.38 (2.75, 6.02) |
7.58 (5.57, 9.58) |
5.20 (3.47, 6.92) |
5.92 (2.86, 8.98) |
|
Protein/ Cr ratio* |
1.89 (0.79, 4.56) |
1.93 (1.35, 2.75) |
3.05 (1.77, 5.25) |
1.79 (1.20, 2.67) |
2.85 (1.74, 4.67) |
1.98 (1.31, 2.97) |
2.67 (1.31, 5.42) |
|
Urine Random Protein* |
177 (31, 1,030) |
246 (139, 434) |
278 (48, 1,612) |
186 (102, 339) |
424 (206, 871) |
185 (107, 321) |
542 (221, 1,327) |
|
GFR* |
111 (79, 158) |
84 (72, 98) |
133 (105, 168)† |
115 (97, 136) |
76 (61, 93)‡ |
108. (94, 125) |
70 (55, 88)‡ |
|
Serum Cr* |
0.56 (0.41, 0.76) |
0.90 (0.78, 1.03)# |
0.60 (0.49, 0.73) |
0.63 (0.55, 0.74) |
0.99 (0.82, 1.20)‡ |
0.66 (0.58, 0.76) |
1.06 (0.85, 1.33)‡ |
|
C3 level* |
55.65 (38.31, 80.84) |
47.77 (40.28, 56.64) |
73.12 (56.51, 94.60)† |
64.28 (53.79, 76.82) |
41.94 (33.44, 52.60)‡ |
53.31 (43.72, 65.01) |
52.35 (37.43, 73.22) |
|
C3 (Low)** |
40.00% |
27.08% |
57.14%† |
47.62% |
15.38%† |
30.00% |
28.57% |
|
C4 level* |
7.42 (4.77, 11.54) |
7.17 (5.86, 8.78) |
12.29 (9.06, 16.66)† |
9.95 (7.92, 12.50) |
6.35 (4.80, 8.41)‡ |
7.63 (6.04, 9.64) |
7.67 (5.17, 11.39) |
|
C4 (Low)** |
20.00% |
12.24% |
52.38%† |
30.95% |
14.81% |
25.00% |
21.43% |
|
DSDNA (Positive)** |
0.00% |
10.64% |
36.84%† |
16.67% |
8.00% |
11.43% |
9.09% |
|
*: Values in the cells are mean (95% CI); |
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Disclosure:
K. Abulaban,
None;
S. P. Ardoin,
None;
M. Klein-Gitelman,
None;
K. A. Rouster-Stevens,
None;
M. Bennett,
None;
L. B. Tucker,
None;
K. Wiley,
None;
S. Nelson,
None;
K. Onel,
None;
N. G. Singer,
None;
B. A. Eberhard,
None;
K. M. O’Neil,
None;
E. B. Brooks,
None;
L. K. Jung,
None;
L. F. Imundo,
None;
T. Wright,
None;
D. Witte,
None;
J. Ying,
None;
P. Devarajan,
None;
H. I. Brunner,
TMA and NIEHS,
9.
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