Download - How do we get the best possible outcome
![Page 1: How do we get the best possible outcome](https://reader036.vdocuments.us/reader036/viewer/2022062315/5681610e550346895dd064cf/html5/thumbnails/1.jpg)
CHILDHOOD SLE IN THE 21ST CENTURY THE STATE OF THE ART
How do we getthe best possible outcome
Thomas J. A. Lehman MDChief, Division of Pediatric rheumatologyHospital for Special Surgery, andProfessor of PediatricsCornell University Medical CollegeNew York, NY
![Page 2: How do we get the best possible outcome](https://reader036.vdocuments.us/reader036/viewer/2022062315/5681610e550346895dd064cf/html5/thumbnails/2.jpg)
CHILDHOOD SLE IN THE 21ST CENTURY THE STATE OF THE ART
The rationale for immunosuppressive therapy:
Prolonged corticosteroid therapy (In excess of 0.25 mg/kg/day)Is associated with an unacceptable frequency of complications
AVN, Cushingoid facies, atherosclerosisSuicide (overt and covert) – no self worth
![Page 3: How do we get the best possible outcome](https://reader036.vdocuments.us/reader036/viewer/2022062315/5681610e550346895dd064cf/html5/thumbnails/3.jpg)
CHILDHOOD SLE IN THE 21ST CENTURY THE STATE OF THE ART
The “standard” cyclophosphamide regimen
1 gm/M2 per dose
7 doses at monthly intervalsFollowed by 10 doses at 3 month intervals
Treat persistent leukopenia with bolus IV solumedrol
Discontinue therapy if Cr > 4.0 after six months of therapy
ALL DOSES GIVEN ON INPATIENT UNIT!!!
![Page 4: How do we get the best possible outcome](https://reader036.vdocuments.us/reader036/viewer/2022062315/5681610e550346895dd064cf/html5/thumbnails/4.jpg)
CHILDHOOD SLE IN THE 21ST CENTURY THE STATE OF THE ART
0
10
20
30
40
50
60
initial 6 moths 12months
18months
36months
48months
60months
P<.05 at 18, 36 and 48 months
ESR
![Page 5: How do we get the best possible outcome](https://reader036.vdocuments.us/reader036/viewer/2022062315/5681610e550346895dd064cf/html5/thumbnails/5.jpg)
CHILDHOOD SLE IN THE 21ST CENTURY THE STATE OF THE ART
0.50.60.70.80.9
11.11.2
initial 6 months 12months
18months
36months
48months
60months
No statistically significant change over 5 yearspatients maintained normal renal function
Cr
![Page 6: How do we get the best possible outcome](https://reader036.vdocuments.us/reader036/viewer/2022062315/5681610e550346895dd064cf/html5/thumbnails/6.jpg)
CHILDHOOD SLE IN THE 21ST CENTURY THE STATE OF THE ART
405060708090
100110120130
initial 6 months 12months
18months
36months
48months
P<.05 at 36 months
CrCl
![Page 7: How do we get the best possible outcome](https://reader036.vdocuments.us/reader036/viewer/2022062315/5681610e550346895dd064cf/html5/thumbnails/7.jpg)
CHILDHOOD SLE IN THE 21ST CENTURY THE STATE OF THE ART
405060708090
100110120
initial 6 months 12months
18months
36months
48months
60months
P<.05 at 6, 12, 18, 36, and 48 months
C3
![Page 8: How do we get the best possible outcome](https://reader036.vdocuments.us/reader036/viewer/2022062315/5681610e550346895dd064cf/html5/thumbnails/8.jpg)
CHILDHOOD SLE IN THE 21ST CENTURY THE STATE OF THE ART
40240440640840
104012401440164018402040224024402640
initial 6 months 12months
18months
36months
48months
P<.05 at 6, 12, and 18 months
24 hr protein
![Page 9: How do we get the best possible outcome](https://reader036.vdocuments.us/reader036/viewer/2022062315/5681610e550346895dd064cf/html5/thumbnails/9.jpg)
CHILDHOOD SLE IN THE 21ST CENTURY THE STATE OF THE ART
05
10152025303540
initial 6 months 12months
18months
36months
48months
60months
P<.05 at 6, 12, 18, 36, 48 and 60 months
Prednisone dosage
![Page 10: How do we get the best possible outcome](https://reader036.vdocuments.us/reader036/viewer/2022062315/5681610e550346895dd064cf/html5/thumbnails/10.jpg)
CHILDHOOD SLE IN THE 21ST CENTURY THE STATE OF THE ART
0
2
4
6
8
10
12
activity 0 activity 36 chronicity 0 chronicity 36
P<. 05 activity decreasedP not significant
chronicity did not increase
![Page 11: How do we get the best possible outcome](https://reader036.vdocuments.us/reader036/viewer/2022062315/5681610e550346895dd064cf/html5/thumbnails/11.jpg)
CHILDHOOD SLE IN THE 21ST CENTURY THE STATE OF THE ART
10
11
12
13
14
initial 6 months 12months
18months
36months
48months
60months
P< .05 at 12, 18, 36 and 48 months
Hb
![Page 12: How do we get the best possible outcome](https://reader036.vdocuments.us/reader036/viewer/2022062315/5681610e550346895dd064cf/html5/thumbnails/12.jpg)
CHILDHOOD SLE IN THE 21ST CENTURY THE STATE OF THE ART
Eight year follow-up15 children completed 3 years of IV cyclophosphamidewith > 96 months of follow-up. 12 males/ 3 females
3 children (20%: 1 female 2 male) developed recurrent diseasewithin one year of completing the three years of treatment.
12 children (80%) remain well, without disease recurrence.
![Page 13: How do we get the best possible outcome](https://reader036.vdocuments.us/reader036/viewer/2022062315/5681610e550346895dd064cf/html5/thumbnails/13.jpg)
CHILDHOOD SLE IN THE 21ST CENTURY THE STATE OF THE ART
Laboratory findings:
Time 0 96 months Paired T
Cr. 0.76 0.73 0.5Hb 10.7 12.9 0.02C3 69 98 0.006C4 10.5 19.1 0.006
SLEDAI 19 3 0.00001
Prednisone 36 13 0.0007
![Page 14: How do we get the best possible outcome](https://reader036.vdocuments.us/reader036/viewer/2022062315/5681610e550346895dd064cf/html5/thumbnails/14.jpg)
CHILDHOOD SLE IN THE 21ST CENTURY THE STATE OF THE ART
Complications
SLE complications
1 patient developed a cavernous sinus thrombosis treated with anticoagulants
No patient developed renal failure, sepsis, or otherlife threatening complications of SLE or therapy
![Page 15: How do we get the best possible outcome](https://reader036.vdocuments.us/reader036/viewer/2022062315/5681610e550346895dd064cf/html5/thumbnails/15.jpg)
CHILDHOOD SLE IN THE 21ST CENTURY THE STATE OF THE ART
Complications:
Two children who received 6 years of cyclophosphamide developed significant complications of therapy
1 amenorrhea1 renal papillary cell carcinoma
![Page 16: How do we get the best possible outcome](https://reader036.vdocuments.us/reader036/viewer/2022062315/5681610e550346895dd064cf/html5/thumbnails/16.jpg)
CHILDHOOD SLE IN THE 21ST CENTURY THE STATE OF THE ART
![Page 17: How do we get the best possible outcome](https://reader036.vdocuments.us/reader036/viewer/2022062315/5681610e550346895dd064cf/html5/thumbnails/17.jpg)
CHILDHOOD SLE IN THE 21ST CENTURY THE STATE OF THE ART
Current therapy for recurrent disease
Children developing active disease following treatmentreceive a 9 month course of intensive immunosuppressionCyclophosphamide 1 gm/M2
Methotrexate 300 mgs/M2
Both given IV monthlyThe MTX 4 hours afterthe cyclophosphamide
Note: Begin with 50 mgs/M2 of MTX and advance as toleratede.g. 50 then 100, then 150, then 300 mgs/M2 in successive months
![Page 18: How do we get the best possible outcome](https://reader036.vdocuments.us/reader036/viewer/2022062315/5681610e550346895dd064cf/html5/thumbnails/18.jpg)
CHILDHOOD SLE IN THE 21ST CENTURY THE STATE OF THE ART
![Page 19: How do we get the best possible outcome](https://reader036.vdocuments.us/reader036/viewer/2022062315/5681610e550346895dd064cf/html5/thumbnails/19.jpg)
CHILDHOOD SLE IN THE 21ST CENTURY THE STATE OF THE ART
Major needs at present:
Standardized criteria for the initiation of therapy
Early intervention PREVENTS BOTH CORTICOSTEROID AND DISEASE RELATED COMPLICATIONS