Session Information
Date: Sunday, October 21, 2018
Title: Pediatric Rheumatology – Clinical Poster I: Lupus, Sjögren’s Disease, and Myositis
Session Type: ACR Poster Session A
Session Time: 9:00AM-11:00AM
Background/Purpose:
Renal disease occurs in 50-70% of all childhood onset systemic lupus erythematosus (cSLE). The prevalence of LN is higher in children and the manifestations are usually more severe than the adult counterparts. Yet there are few reports on long term outcomes in cSLE patients with LN. This study aimed to study long-term outcomes in Chinese cSLE patients with biopsy proven LN.
Methods:
We completed retrospective chart reviews of all patients diagnosed and followed with SLE and biopsy proven LN from Jan 2000 to Feb 2018 in 3 regional hospitals in Hong Kong. All the patients met either the ACR or SLICC classification criteria (after year 2012) for SLE. Patients with LN were classified according to WHO criteria or INS/RPS criteria (after year 2003). The outcomes including chronic kidney disease (CKD) stages, dialysis, renal transplant and death were retrieved from patient charts at 6 time points: the year of diagnosis (Y0), one year after diagnosis (Y1), Y5, Y10, Y15 and last follow up (Y last) of patients.
Results:
Our cohort included 104 cSLE patients with biopsy proven LN (Table 1). Majority (85.6%) had LN diagnosed within 2 years of SLE diagnosis. Thirty two patients had second renal biopsies. None of the Class V LN transformed to proliferative LN in their second renal biopsies and 9 (28.1%) transformed from non-proliferative to proliferative LN with a mean of 5.87 years from the 1st to 2nd renal biopsy. There were total 144 biopsies in the study cohort period. Cyclophosphamide (CYC) was used as induction therapy in 38/99 (38.38%) of proliferative LN (Class III/IV +/- V) episodes in 36 patients vs Mycophenolate mofetil (MMF) in 28/99 (28.3%) episodes in 28 patients. Three patients required transient dialysis during the course and none required long term dialysis. Two patients had renal transplant and 5 patients died. The outcomes of death and renal transplant are worst in patients with proliferative LN without using either CYC or MMF as induction therapy and are best in non-proliferative LN as expected (Table 2 and 3).
Conclusion:
The renal outcome of LN in cSLE is suboptimal with close to 30% of patients have significant proteinuria and 18.2% have abnormal renal function (CKD stage 2 or above) at their last visit. Patients with non-proliferative LN have best outcomes followed by proliferative LN treated with CYC or MMF in the induction phase.
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Table 1. Basic demographic and distribution of classes of LN
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Feature |
Value or count |
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Sex · Male · Female |
Male 11 (10.6%) Female 93 (89.4%) |
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Mean/median age of diagnosis of SLE (years) |
12.98/13 (IQR 5, SD 3.39) |
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Mean/median age of diagnosis of LN (years) |
13.7/14.5 (IQR 4.75, SD 3.22) |
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Mean/median duration of follow up (years) |
8.66/9 (IQR 7, SD 4.55) |
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Histologic class on first renal biopsy n (%) Total N = 104 |
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|
· II · III · IV · V · II+III · II+V · III+V · IV+V · VI · Unable to classify |
8 (7.69) 20 (19.23) 35 (33.65) 8 (7.69) 5 (4.80) 11(10.58) 10 (9.62) 5 (4.81) 1 (0.96) 1 (0.96) |
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Histologic class on second renal biopsy n (%) Total N = 32 |
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· II · III · IV · V · II+V · III+V · IV+V |
1 (3.13) 5 (15.63) 7 (21.88) 4 (12.5) 6 (18.75) 5 (15.63) 4 (12.5) |
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Table 2. Outcome of non-proliferative and proliferative LN. |
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Non-proliferative LN N = 20 |
Proliferative LN |
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CYC induction (per 100-patient years) N=36 |
MMF induction (per 100-patient years) N = 28 |
Non CYC or MMF (per 100-patient years) N=20 |
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CKD stage >= 4 at Y last |
0 |
0.835 |
0.971 |
0 |
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Nephrotic range of proteinuria at Y last |
0.613 |
0.557 |
1.456 |
0 |
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Transient dialysis |
0 |
0.557 |
0 |
0.658 |
|
Renal transplant |
0 |
0 |
0.485 |
*0.658 |
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Death |
0 |
0.835 |
0 |
1.316 |
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*same patient with renal transplant
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Table 3. Outcome at different time points. |
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CKD stage and degree of proteinuria |
Y0 (n=104) |
Y1 (n = 104) |
Y5 (n=82) |
Y10 (n = 44) |
Y15 ( n =16) |
Y last (n = 99) |
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Normal (eGFR >90 ml/min/1.73m2) |
55 |
86 |
69 |
31 |
9 |
81 |
|
2 (eGFR 60-89 ml/min/1.73m2) |
25 |
13 |
8 |
6 |
1 |
12 |
|
3 (eGFR 30-59ml/min/7.73m2) |
17 |
2 |
3 |
3 |
2 |
2 |
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4 (eGFR 15-29 ml/min/1.73m2) |
5 |
2 |
1 |
2 |
1 |
3 |
|
5 (eGFR <15 ml/min/1.73m2) |
0 |
0 |
0 |
0 |
1 |
1 |
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Significant proteinuria UPCr ratio >500mg/g but <=2000mg/g |
35 |
25 |
13 |
7 |
4 |
23 |
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Nephrotic proteinuria UPCr ratio >2000mg/g |
60 |
6 |
3 |
4 |
1 |
6 |
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Cumulative death |
0 |
0 |
1 (censored) |
2 (censored) |
4 (censored) |
5 (censored) |
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Cumulative renal transplant |
0 |
0 |
1 (renal transplant at Y4) |
1 |
2 (one renall transplant at Y 13)
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2 (both alive at CKD stage 2) |
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Missing data |
2
|
1
|
1
|
2
|
No |
No |
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Y (n): number of year reference to the date of first renal biopsy which confirmed lupus nephritis e.g Y1: one year after diagnosis of LN; UPCr ratio: Urine protein to creatinine ratio
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To cite this abstract in AMA style:
Chiang G, Wong SN, Law C, Lee KP, Szeto CC, Mok CC, Tam LS, Leung TF. The Long Term Outcomes in Chinese Children Diagnosed with Systemic Lupus Erythematosus and Biopsy Proven Lupus Nephritis – a 18-Year Cohort [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/the-long-term-outcomes-in-chinese-children-diagnosed-with-systemic-lupus-erythematosus-and-biopsy-proven-lupus-nephritis-a-18-year-cohort/. Accessed .« Back to 2018 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-long-term-outcomes-in-chinese-children-diagnosed-with-systemic-lupus-erythematosus-and-biopsy-proven-lupus-nephritis-a-18-year-cohort/