Session Information
Session Type: Abstract Submissions (ACR)
Background/Purpose: Little is known about the long-term morbidity and mortality of childhood-onset SLE (cSLE) after transition to adult care; however, linking clinical data to administrative databases enables study of previously unknown outcomes. Our objectives were to: i) describe differences in cSLE phenotype over time; ii) determine the mortality rate; and iii) describe health care utilization trends.
Methods: A retrospective chart review of all cSLE patients (<18 years at diagnosis) diagnosed between Jan 1, 1984 and Dec 31, 2011 and followed for ≥ 1 year was conducted after contacting all pediatric and adult rheumatologists and nephrologists practicing in Ontario. Clinical data and Ontario Health Insurance Plan (OHIP) numbers were securely transferred to the Institute for Clinical Evaluative Sciences (ICES). OHIP numbers were transformed into an encrypted ICES key number (IKN) used to link the cohort to multiple administrative datasets to determine the outcomes of interest. Patients were stratified by era of diagnosis (1984 – 1989; 1990 – 2001; 2002 – 2011) because of available data on income and utilization, and to examine trends as immunosuppressive treatments changed over time. Means ± standard deviations are given unless noted.
Results: IKN linkage was successful for 620 cSLE patients in this inception cohort. The cohort was predominantly female (81%) with follow-up of 10.7 (± 7.3) years, representing 6629 person-years of disease. Age at diagnosis was 12.8 ± 3.2, which was similar across eras. Based on postal codes, patients were evenly distributed among the income quintiles; 18% were in the lowest and 13% in the highest quintile. For the whole cohort, self-reported ethnicity was White 42%, Asian 24%, Black 15%, South Asian 13%, Aboriginal 1.3% and Other 4%; however there is a larger percentage of non-white patients in the most recent era (61%), likely reflecting immigration patterns. See Table for differences in distribution of clinical features over time. There were 23 deaths for a crude mortality rate 3.7%, and standardized mortality rate of 2.8%. Six deaths (26%) occurred in the first year following cSLE diagnosis, and 14 (61%) after transfer to adult care. Physician office visits were frequent within the first year after diagnosis, with 23% requiring ≥ 20 visits, and 46% with 10 – 19 physician visits. Moreover, in the first year 43% of patients had at least one emergency room visit, 38% were hospitalized at least once, and 12% required ≥ 3 hospitalizations.
Table: Clinical Features by Era of Diagnosis (*cell size <6 suppressed)
|
|
cSLE Cohort (N=620) |
Era of Diagnosis |
p-value |
||
|
1984 – 1989 (N=52) |
1990 – 2001 (N=194) |
2002 – 2011 (N=374) |
|||
|
Disease Duration (y) |
10.7 ± 7.3 |
25.7 ± 3.4 |
16.0 ± 3.9 |
5.8 ± 2.7 |
|
|
Malar Rash (N, %) |
485 (78) |
44 (85) |
155 (80) |
286 (77) |
0.32 |
|
Discoid Rash |
|
<6* |
12 (6) |
11 (3) |
0.18 |
|
Oral/Nasal Ulcers |
240 (39) |
24 (46) |
87 (45) |
129 (35) |
0.03 |
|
Photosensitivity |
232 (37) |
26 (50) |
85 (44) |
121 (32) |
0.004 |
|
Coombs+ Anemia |
178 (29) |
22 (42) |
55 (28) |
101 (27) |
<0.001 |
|
Leukopenia |
118 (19) |
10 (19) |
29 (15) |
79 (21) |
0.20 |
|
Lymphopenia |
341 (55) |
34 (65) |
108 (56) |
199 (53) |
0.25 |
|
Thrombocytopenia |
186 (30) |
13 (25) |
70 (36) |
103 (28) |
0.08 |
|
Arthritis |
449 (72) |
41 (79) |
137 (70) |
271 (73) |
0.50 |
|
Pleuritis |
96 (16) |
9 (17) |
39 (20) |
48 (13) |
0.07 |
|
Pericarditis |
85 (14) |
17 (33) |
37 (19) |
31 (8) |
<0.001 |
|
Nephritis (by biopsy) |
273 (44) |
36 (69) |
107 (55) |
130 (35) |
<0.001 |
|
Psychosis |
|
<6* |
13 (7) |
41 (11) |
0.23 |
|
Seizures |
|
<6* |
9 (5) |
12 (3) |
0.27 |
|
ANA |
608 (98) |
51 (98) |
191 (99) |
366 (98) |
0.89 |
|
dsDNA |
441 (71) |
44 (85) |
166 (86) |
231 (62) |
<0.001 |
|
ENA (Sm, Ro, La, RNP) |
426 (69) |
38 (73) |
146 (75) |
242 (65) |
0.03 |
|
Antiphospholipid Abs |
263 (43) |
9 (17) |
102 (53) |
152 (41) |
<0.001 |
|
Thromboembolism |
|
<6* |
16 (8) |
13 (4) |
0.04 |
Conclusion: The prevalences of autoantibodies and certain clinical manifestations such as lupus nephritis are declining in more recently diagnosed cSLE patients. These differences may reflect changes in cSLE over time, earlier diagnosis or a shifting ethnicity distribution in Ontario. For cSLE, the mortality rate is highest in the first year following diagnosis, and overall health care utilization is high.
Disclosure:
D. M. Levy,
None;
N. Gunraj,
None;
J. E. Pope,
None;
J. C. Thorne,
None;
W. Fidler,
None;
P. B. Dent,
None;
J. Roth,
None;
R. A. Berard,
None;
M. Berall,
None;
A. Guttmann,
None;
E. D. Silverman,
None.
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