Session Information
Date: Monday, October 22, 2018
Title: Systemic Lupus Erythematosus – Clinical Poster II: Biomarkers and Outcomes
Session Type: ACR Poster Session B
Session Time: 9:00AM-11:00AM
Background/Purpose:
A new classification criteria for SLE was proposed at the ACR/ARHP 2017 annual meet. The aim of this study was to compare the performance of the proposed ACR/EULAR’17 criteria against the 2012 SLICC (SLICC’12) and ACR 1997 (ACR’97) classification criteria for SLE in the adult (A-SLE) and juvenile SLE (J-SLE) cohort from India.
Methods:
Patient records were chosen from the disease registry collected over the past 8 years for A-SLE, J-SLE, MCTD, Primary SS and primary APS from a tertiary care center in South India. The records were screened to include only ANA associated rheumatic diseases (AARD). Patients who were diagnosed as SLE at their first visit by trained rheumatologists were designated as cases and the other rheumatic diseases as controls. The performance of ACR 97, SLICC’12 and ACR/EULAR’17 criteria at the first visit was analysed. Sensitivity of the three criteria were analysed by sub classifying the patients based on the duration of illness as <18, 19-36, 37-54 and >54 months.
Results:
We analysed 273 j-SLE and 884 a-SLE patients as cases and 435 other AARDs (MCTD-119, UCTD-69, SS-217, APS-30) as controls. The demography, clinical and immunological profile of the cases and controls are shown in table 1.
The sensitivity, specificity, positive and negative predictive values of each of these criteria are depicted in table 2. The subgroup of adult patients with <54 months disease had the largest difference in sensitivity between the ACR’97 and the other two criteria; whereas the difference was seen in the j-SLE patients presenting within 18 months of disease onset (table 3). False positivity was seen in 41, 71 and 66 patients in ACR’97, SLICC’12 and ACR/EULAR’17 criteria respectively. A score of +4 for low serum c3 and c4 led to the false diagnosis of 15 controls by the ACR/EULAR’17 who were not diagnosed by ACR’97 criteria.
Conclusion:
The proposed ACR/EULAR ’17 criteria has higher sensitivity and lower specificity than the ACR’97 criteria but is similar to the SLICC’12 criteria among J-SLE and A-SLE. A score of +4 for low c3 and c4 was the probable cause for lower specificity and needs consideration. Assigning an appropriate negative or positive score in case of negative or positive antibodies to either of anti-nucleosome, anti-ribosomal p or anti-histone in all the anti-dsDNA and anti-Smith negative patients may overcome this shortcoming.
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Table 1: Demography, Clinical and immunological profile of patients with juvenile SLE, Adult SLE and other ANA associated rheumatic diseases. |
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Juvenile SLE (N=273) |
Adult SLE (N=884) |
Controls (N= 435) |
|
Age (Mean ± SD, In Years) |
18.45 ± 7.48 |
31.46 ± 9.29 |
38.23 ± 12.55 |
|
Duration of Illness (Median, IQR, In Months) |
12 (4,25) |
12 (5,16) |
36 (12,72) |
|
Gender (M:F) |
32:241 |
53:831 |
14: 421 |
|
Photosensitivity |
67 (24.5) |
242 (27.4) |
27 (6.2) |
|
Malar Rash |
134 (49.1) |
435 (49.2) |
5 (1.1) |
|
ACLE |
149 (54.6) |
488 (55.2) |
19 (4.4) |
|
SCLE |
7 (2.6) |
25 (2.8) |
3 (0.7) |
|
DLE |
46 (16.8) |
148 (16.7) |
7 (1.6) |
|
Non-Scarring Alopecia |
106 (38.8) |
318 (36.0) |
14 (3.2) |
|
Oral Ulcer |
128 (46.9) |
387 (43.8) |
19 (4.4) |
|
Arthritis |
149 (54.6) |
547 (61.9) |
229 (52.6) |
|
Serositis |
34 (12.5) |
143 (16.2) |
11 (2.5) |
|
Acute pericarditis |
9 (3.3) |
29 (3.3) |
0 (0) |
|
Class III/IV LN |
45 (16.5) |
157 (17.8) |
0 (0) |
|
Class II/ V LN |
10 (3.7) |
51 (5.8) |
1 (0.2) |
|
Neurological disorder |
61 (22.3) |
177 (20) |
6 (1.4) |
|
Autoimmune hemolytic anemia |
55 (20.1) |
147 (16.6) |
3 (0.7) |
|
Leucopenia |
78 (28.6) |
236 (26.7) |
27 (6.2) |
|
Lymphopenia |
79 (28.9) |
241 (27.3) |
13 (3.0) |
|
Thrombocytopenia |
74 (27.1) |
165 (18.7) |
20 (4.6) |
|
Fever |
103 (37.7) |
264 (29.9) |
6 (1.4) |
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Anti dsDNA positivity |
120 (43.9) |
450 (50.9) |
26 (6.0) |
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Lupus Anticoagulant / ACLA/Beta 2 GP1 positivity |
98 (35.9) |
47 (5.3) |
59 (13.6) |
|
C3 or C4 low |
125 (45.8) |
393 (44.5) |
30 (6.9) |
|
Both C3 and C4 low |
72 (26.4) |
219 (24.8) |
16 (3.7) |
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Positive coombs test |
55 (20.1) |
167 (18.9) |
33 (7.6) |
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Table 2: Performance of each classification criteria in patients with SLE |
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Juvenile SLE |
Adult SLE |
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Criteria |
ACR 1997 |
SLICC 2012 |
ACR 2017 |
ACR 1997 |
SLICC 2012 |
ACR 2017 |
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Sensitivity (CI) |
80.95 (75.78-85.43) |
97.07 (94.31- 98.73) |
92.67 (88.91-95.47) |
82.13 (79.44- 84.60) |
95.59 (94.02- 96.84) |
92.31 (90.35- 93.98) |
|
Specificity (CI) |
90.80 (87.69- 93.35) |
83.68 (79.86- 87.03) |
85.29 (81.60- 88.48) |
90.80 (87.69-93.35) |
83.68 (79.86- 87.03) |
85.29 (81.60- 88.48) |
|
Positive predictive value (CI) (As calculated in study cohort) |
84.67 (80.35- 88.19) |
78.87 (75.09- 82.21) |
79.81 (75.87-83.25) |
94.78 (93.10- 96.07) |
92.25 (90.58- 93.64) |
92.73 (91.04- 94.12) |
|
True PPV (Assuming Prevalence = 3.2/1,00,000) |
0.01 (0.01-0.02) |
0.02 (0.02-0.02) |
0.02 (0.02-0.02) |
0.03 (0.02- 0.03) |
0.02 (0.02-0.02) |
0.02 (0.02-0.02) |
|
Negative predictive value (CI) (As calculated in study cohort) |
88.37 (85.59-90.67) |
97.85 (95.83-98.90) |
94.88 (92.39-96.59) |
71.43 (68.39- 74.28) |
90.32 (87.26- 92.71) |
84.51 (81.23- 87.31) |
|
True NPV (Assuming Prevalence = 3.2/1,00,000) |
100 (100- 100) |
100 (100- 100) |
100 (100- 100) |
100 (99.99-100) |
100 (100- 100) |
100 (100- 100) |
|
Positive Likelihood Ratio |
8.80 (6.52-11.89) |
5.95 (4.8-7.36) |
6.30 (5.01-7.92) |
8.93 (6.64- 12.02) |
5.86 (4.73-7.25) |
6.27 (5.00- 7.87) |
|
Negative Likelihood Ratio |
0.21 (0.16- 0.27) |
0.04 (0.02-0.07) |
0.09 (0.06- 0.13) |
0.20 (0.17- 0.23) |
0.05 (0.04- 0.07) |
0.09 (0.07-0.11) |
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Diagnostic Odds ratio (CI) |
41.96 (26.92-65.41) |
169.82 (80.38-358.76) |
73.33 (43.29- 124.20) |
45.37 (31.40- 65.55) |
111.08 (73.75- 67.29) |
70.60 (49.06- 101.58) |
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Table 3: Sensitivity for each classification criteria for groups with different disease durations
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Sensitivity of ACR 97 |
Sensitivity of SLICC 2012 |
Sensitivity of ACR/EULAR 2017 |
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Adult SLE |
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<18 months (n=539) |
81.08 (77.51- 84.30) |
95.36 (93.23- 96.98) |
92.57 (90.01- 94.64) |
|
19-36 months (n=164) |
87.20 (81.09- 91.90) |
96.95 (93.03- 99.0) |
95.73 (91.40-98.27) |
|
37-54 months (n=65) |
72.31 (59.81- 82.69) |
90.77 (80.98- 96.54) |
89.23 (79.06- 95.56) |
|
>54 months (n=114) |
85.96 (78.21- 91.76) |
97.37 (92.50- 99.45) |
89.47 (82.33- 94.44) |
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Juvenile SLE |
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<18 months (n=183) |
78.69 (72.04- 84.38) |
96.72 (93.0-98.79) |
91.80 (86.84-95.34) |
|
19-36 months (n=35) |
88.57 (73.26- 96.80) |
100 (90-100) |
94.92 (80.84- 99.30) |
|
37-54 months (n=11) |
100 (71.51-100) |
100 (71.51-100) |
100 (71.51-100) |
|
>54 months (n=44) |
79.55 (64.70-90.20) |
95.45 (84.53- 99.44) |
93.18 (81.34-98.57) |
To cite this abstract in AMA style:
KG C, Kumar G, R P S, Negi VS. Performance of the Proposed American College of Rheumatology / European League Against Rheumatism 2017 Classification Criteria for SLE in Adult and Juvenile Systemic Lupus Erythematosus and Other Anti-Nuclear Antibody Related Rheumatic Diseases [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/performance-of-the-proposed-american-college-of-rheumatology-european-league-against-rheumatism-2017-classification-criteria-for-sle-in-adult-and-juvenile-systemic-lupus-erythematosus-and-other-anti/. Accessed .« Back to 2018 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/performance-of-the-proposed-american-college-of-rheumatology-european-league-against-rheumatism-2017-classification-criteria-for-sle-in-adult-and-juvenile-systemic-lupus-erythematosus-and-other-anti/