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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. The rationale for immunosuppressive therapy:.

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How do we get the best possible outcome

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  1. 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

  2. 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

  3. 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!!!

  4. ESR P<.05 at 18, 36 and 48 months

  5. Cr No statistically significant change over 5 years patients maintained normal renal function

  6. CrCl P<.05 at 36 months

  7. C3 P<.05 at 6, 12, 18, 36, and 48 months

  8. 24 hr protein P<.05 at 6, 12, and 18 months

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

  10. P not significant chronicity did not increase P<. 05 activity decreased

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

  12. 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.

  13. 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

  14. 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

  15. Complications: Two children who received 6 years of cyclophosphamide developed significant complications of therapy 1 amenorrhea 1 renal papillary cell carcinoma

  16. 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

  17. Major needs at present: Standardized criteria for the initiation of therapy Early intervention PREVENTS BOTH CORTICOSTEROID AND DISEASE RELATED COMPLICATIONS

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