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How do we get the best possible outcome. Thomas J. A. Lehman MD Chief, Division of Pediatric rheumatology Hospital for Special Surgery, and Professor of Pediatrics Cornell University Medical College New York, NY. The rationale for immunosuppressive therapy:.

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How do we get the best possible outcome

How do we get

the best possible outcome

Thomas J. A. Lehman MD

Chief, Division of Pediatric rheumatology

Hospital for Special Surgery, and

Professor of Pediatrics

Cornell University Medical College

New York, NY


How do we get the best possible outcome

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, atherosclerosis

Suicide (overt and covert) – no self worth


How do we get the best possible outcome

The “standard” cyclophosphamide regimen

1 gm/M2 per dose

7 doses at monthly intervals

Followed 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!!!


How do we get the best possible outcome

ESR

P<.05 at 18, 36 and 48 months


How do we get the best possible outcome

Cr

No statistically significant change over 5 years

patients maintained normal renal function


How do we get the best possible outcome

CrCl

P<.05 at 36 months


How do we get the best possible outcome

C3

P<.05 at 6, 12, 18, 36, and 48 months


How do we get the best possible outcome

24 hr protein

P<.05 at 6, 12, and 18 months


How do we get the best possible outcome

Prednisone

dosage

P<.05 at 6, 12, 18, 36, 48 and 60 months


How do we get the best possible outcome

P not significant

chronicity did not increase

P<. 05 activity decreased


How do we get the best possible outcome

Hb

P< .05 at 12, 18, 36 and 48 months


How do we get the best possible outcome

Eight year follow-up

15 children completed 3 years of IV cyclophosphamide

with > 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.


How do we get the best possible outcome

Laboratory findings:

Time 0

96 months

Paired T

Cr.

0.76

0.73

0.5

Hb

10.7

12.9

0.02

C3

69

98

0.006

C4

10.5

19.1

0.006

SLEDAI

19

3

0.00001

Prednisone

36

13

0.0007


How do we get the best possible outcome

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


How do we get the best possible outcome

Complications:

Two children who received 6 years of cyclophosphamide

developed significant complications of therapy

1 amenorrhea

1 renal papillary cell carcinoma


How do we get the best possible outcome

Current therapy for recurrent disease

Children developing active disease following treatmentreceive a 9 month course of intensive immunosuppression

Both given IV monthly

The MTX 4 hours afterthe cyclophosphamide

Cyclophosphamide 1 gm/M2Methotrexate 300 mgs/M2

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


How do we get the best possible outcome

Major needs at present:

Standardized criteria for the initiation of therapy

Early intervention PREVENTS BOTH CORTICOSTEROID

AND DISEASE RELATED COMPLICATIONS