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New Onset “Autoimmune Hepatitis“ in Liver Pediatric Recipients

New Onset “Autoimmune Hepatitis“ in Liver Pediatric Recipients.

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New Onset “Autoimmune Hepatitis“ in Liver Pediatric Recipients

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  1. New Onset “Autoimmune Hepatitis“ in Liver Pediatric Recipients

  2. A. SONZOGNI , M. SPADA , S. RIVA,M. COLLEDAN , A. LUCIANETTI, A. SEGALIN, A. BERTANI, M. L. MELZI , B. GRIDELLI LIVER TRANSPLANTATION CENTER, OSPEDALI RIUNITI , BERGAMO , ITALYA. J. DEMETRISTRANSPLANTATION PATHOLOGY UPMC,PITTSBURGH PA , USAM. MINERVINIISMETT , PALERMO , ITALY

  3. INTRODUCTION - de novo “autoimmune” hepatitis is a major problem in pediatric liver transplantation - appearance of autoantibodies is associated with a wide spectrum of clinical / histological features

  4. AIM OF THE STUDY To investigate the impact of “autoimmune hepatitis” as long-term complication of pediatric OLT and links with other post-operative diseases

  5. MATERIALS • median age at tx 1.8 yrs. (0.2-17 yrs.) • median follow-up 1.5 yrs. (0.2 - 10 yrs.) 113 liver tx in 102 pts

  6. NATIVE DISEASES

  7. IMMUNOSUPPRESSION • CYCLOSPORIN 58 • TACROLIMUS 29 • SWITCH CYCLO - TACRO 15

  8. OLT & AUTOANTIBODIES 16 / 102 pts ( 15 % ) • median age at tx 3.7 yrs ( 0.7 -15 yrs.) • median follow-up 1.5 yrs. ( 0.4 - 10 yrs.) • median time from OLT 3.6 yrs (0.2-9.9yrs.)

  9. TYPE OF GRAFT • WHOLE LIVER7 • REDUCED LIVER II-III SEG.4 • IN SITU SPLIT II-III SEGM.5

  10. IMMUNOSUPPRESSION IN PTS. W/AUTOANTIBODIES • CYCLOSPORIN 8 • TACROLIMUS 3 • SWITCH CYCLO-TACROLIMUS 5

  11. TYPE OFAUTOANTIBODIES 3 ANA + ASMA 5 ANA 4 ASMA 1 ASMA+ ANA + ENA 1 ASMA + cANCA 2 LKM

  12. CLINICAL PRESENTATION • median ALT 145 U./l. (n.v. up to 45 U./l. ) range 45 - 350 • median IgG 1400 mg. / dl. ( n.v. 310 - 160 mg./ dl. ) range 600 - 2170

  13. AUTOIMMUNE HEPATITIS SCORE * * J. Hepatology 31: 929-938, 1999

  14. CLINICAL PRESENTATION IN 7 / 16 PATIENTS LIVER FUNCTION TEST ALTERATIONS PRECEEDED APPEARANCE OF AUTOANTIBODIES MEDIUM LAG TIME 5.5 MONTHS ( 3-10 MONTHS)

  15. PREVIOUS POST-OLT COMPLICATIONS • ACR ( 4 ) • biliary strictures ( 1 ) • ACR & polisierositis ( 1 ) • ACR & biliary strictures ( 2 ) • ACR & colic stricture ( 1 ) • ACR and portal thrombosis ( 1 ) • chronic varicella virus infection (1)

  16. CLINICAL FOLLOW - UP NO THERAPY 10 PERSISTENCE OF AUTOAB’S 7 DISAPPAEARANCE OF AUTOAB’S 3 STEROIDS THERAPY 2 PERSISTENCE OF AUTOAB’S 2 STEROIDS & AZOTHIOPRINE 4 PERSISTENCE OF AUTOAB’S 4

  17. HISTOLOGICAL FOLLOW-UP NO THERAPY 1 1 UNCHANGED STEROIDS & AZOTHIOPRINE 4 2 UNCHANGED * 2 IMPROVED 1 ( 4 TO 2 HAI ) 1 ( 5 TO 2 HAI ) * 1 pt. w/autoab’s disapparearance

  18. unknown EBV status 9 follow-up <1 month 9 medium follow-up 1.5 yrs known EBV status 68 EBNA-Ig +ve 32 (47 %) EBNA-Ig -ve 36 (53 %) EBV - STATUS PTS W/OUT AUTOAB PTS W/AUTOAB • unknown EBV status 3 • medium follow-up 3 yrs • known EBV status 13 EBNA-Ig +ve 3 (23 %) EBNA-Ig -ve 10 (77 %)

  19. EBV INFECTION POST - OLT NO AUTOAB AUTOAB

  20. ITEMS TO BE FURTHER INVESTIGATED • What is incidence of the disease ? • What is its outcome ? • Which is its treatment ?

  21. ITEMS TO BE FURTHER INVESTIGATED • Are there any links with post-OLT infectious diseases ? • Are there any links with native pathology ?

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