Evaluation of patients with autoimmune hepatitis: eleven years of experience

Öz

Aim: This study aimed to review a series of patients with autoimmune hepatitis in terms of disease pattern nbsp; laboratory results treatment outcomes and adverse effects of treatment Material and Method: Children with autoimmune hepatitis were retrospectively reviewed In all patients viral and metobolic eitologies were excluded Patients were classified as type 1 type 2 and non classified type as well as acute and chronic groups Treatment response and cessation of treatment were evaluated Results: Patients were beetween 4 and 17 years old 12 plusmn;2 68 years Twenty patients were female 11 patients were male nbsp; Seventeen patients were in acute and 14 patients were in chronic group; 18 of them were in type 1 6 of them in type 2 and 7 of them were in non classified type nbsp; Deflazacort was started in all patients Azathioprine 2 mg kg day was added to 10 patients with late response at the end of the third month Deflazacort dosage was decreased at 6 8 weeks intervals and continued at a maintanance dosage of 5 mg day After a two year period complete response was obtained in 24 partial response was obtained in 5 patients No response was obtained in 2 patients In the nine year follow up period treatment was ended in 6 patients In one patient there was a recurrance in 6 months; the remaining 5 patients are still being followed up without a problem Despite treatment portal hypertension was observed in 5 patients Conclusions: Early diagnosis and treatment of childhood autoimmune hepatitis can decrease the risk of progression to cirrhosis and can increase the survival Deflazocort can be a choice nbsp; instead of prednisolone because of lesser side effects and efficiancy in treatment Turk Arch Ped 2012; 47: 28 32

Anahtar Kelimeler

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Otoimmün hepatitli olgularımızın irdelenmesi: 11 yıllık deneyimimiz

Öz

Amaç: Otoimmün hepatit tanılı olgularımızın klinik ve laboratuvar verilerini tedavi sonuçlarını ve nbsp; tedavi yan etkilerini irdelemeyi amaçladık Gereç ve Yöntem: Otoimmün hepatit tanılı olguların dosyaları geriye dönük olarak incelendi nbsp; Tüm olgularda viral ve metabolik nedenler dışlandı Olgular tip 1 tip 2 tanımlanamayan tip ve ayrıca akut kronik olarak gruplandırıldı Olguların tedavi yanıtı ve tedavi sonlandırılma bilgileri değerlendirildi. 

Bulgular: Olgular 4 17 yaş 12 plusmn;2 68 yıl arasında olup 20 rsquo;si kız 11 rsquo;i erkekti Olguların 17 rsquo;si akut 14 rsquo;ü kronik; 18 rsquo;i tip 1 altısı tip 2 ve yedisi tanımlanamayan tip idi Tüm olgulara deflazakort başlandı Geç yanıt alınan 10 olguya üçüncü ayın sonunda azatiyopirin 2 mg kg gün eklendi Deflazakort 6 8 haftada bir azaltılarak 5 mg gün dozuyla devam edildi İki yılın sonunda 24 olgu tam yanıtlı beş olgu kısmi yanıtlı iki olgu ise yanıtsız olarak değerlendirildi Dokuz nbsp; yıllık izlem süresince altı olgunun nbsp; tedavisi sonlandırıldı Bir olguda nbsp; altı ay nbsp; sonra tekrarlama oldu Tedavisi kesilen nbsp; beş olgu hala sorunsuz olarak izlenmektedir Tedaviye rağmen beş olguda portal hipertansiyon bulguları gelişti.

Çıkarımlar: Çocukluk yaşı otoimmün hepatitinin erken tanı ve tedavisi siroza ilerleyiş hızını azaltmakta ve yaşam süresini uzatmaktadır Deflazakortun tedavideki etkinliği ve yan etkilerinin azlığı göz önüne alındığında otoimmün hepatit tanılı çocuklarda kullanılabileceği görüşündeyiz.

Anahtar Kelimeler

• Azatiyopirin
• çocuk
• deflazakort
• otoimmün hepatit

Kaynakça

1. Oettinger R, Brunnberg A, Gerner P, Wintermeyer P, Jenke A, Wirth S.
2. Clinical features and biochemical data of Caucasian children at diagnosis of autoimmune hepatitis. J Autoimmun 2005; 24: 79-84.
3. Mackay IR. Historical reflections on autoimmune hepatitis. World J
4. Gastroenterol 2008; 14: 3292-300.
5. Vergani D, Mieli-Vergani G. Aetiopathogenesis of autoimmune
6. hepatitis. World J Gastroenterol 2008; 14(21): 3306-12.
7. Czaja AJ. Autoimmune hepatitis: evolving concepts and treatment
8. strategies. Dig Dis Sci 1995; 40: 435-56.

9. Z Ben-Ari, Czaja AJ. Autoimmune hepatitis and variant forms. Gut 2001; 49: 589-94.
10. Czaja AJ. Clinical features, differential diagnosis and treatment of
11. autoimmune hepatitis in the elderly. Drugs Aging 2008; 25: 219-39.
12. Czaja AJ, Doherty DG, Donaldson PT. Genetic bases of autoimmune
13. hepatitis. Dig Dis Sci 2002; 47: 2139-50.
14. Zachou K, Rigopoulou E, Dalekos GN. Autoantibodies and
15. autoantigens in autoimmune hepatitis: important tools in clinical practice and to study pathogenesis of the disease. J Autoimmune Dis 2004; 15: 1-2.
16. Djilali-Saiah I, Fakhfakh A, Louafi H, Caillat-Zucman S, Debray D,
17. Alvarez F. HLA class II influences humoral autoimmunity in patients with type 2 autoimmune hepatitis. J Hepatol 2006; 45: 844- 50.
18. Ma Y, Bogdanos DP, Hussain MJ, et al. Polyclonal T-cell responses to cytochrome P450IID6 are associated with disease activity in autoimmune hepatitis type 2. Gastro 2006; 130: 868-82.
19. Bellomo-Brandão MA, Costa-Pinto EA, De Tommaso AM, Hessel G. Clinical and biochemical features of autoimmune hepatitis in 36 pediatric patients. Arq Gastroenterol 2006; 43: 45-9.
20. Alvarez F, Berg PA, Bianchi FB, et al. International Autoimmune Hepatitis Group report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol 1999; 31: 929-38.
21. Czaja AJ. Performance parameters of the diagnostic scoring systems for autoimmune hepatitis. Hepatology 2008; 48: 1540-8.
22. Knodell RG, Ishak KG, Black WC, et al. Formulation and application of a numerical scoring system for assessing histological activity in asymptomatic chronic active hepatitis. Hepatology 1981; 1: 431-5.
23. Desmet VJ, Gerber M, Hoofnagle JH, Manns M, Scheuer PJ. Classification of chronic hepatitis: diagnosis, grading and staging. Hepatology 1994; 19: 1513-20.
24. Fallatah HI, Akbar HO, Qari YA. Autoimmune hepatitis: single-center experience of clinical presentation, response to treatment and prognosis in Saudi Arabia. Saudi J Gastroenterol 2010; 16: 95-9.
25. Gregorio GV, Portmann B, Reid F, et al. Autoimmune hepatitis in childhood: a 20-year experience. Hepatology 1997; 25: 541-7.
26. Ozen H, Koçak N, Saltik IN, Yüce A, Gürakan F. Autoimmune hepatitis. Indian J Pediatr 2001; 68: 725-8.
27. Rafeey M, Kianrad M, Hasani A. Autoimmune hepatitis in Iranian children. Indian J Gastroenterol 2007; 26: 11-3.
28. Maggiore G, Veber F, Bernard O, et al. Autoimmune hepatitis associated with anti-actin antibodies in children and adolescents. J Pediatr Gastroenterol Nutr 1993; 17: 376-81.
29. Choudhuri G, Somani SK, Baba CS, Alexander G. Autoimmune hepatitis in India: profile of an uncommon disease. BMC Gastroenterol 2005; 5: 27.
30. Porta G, Miura IK, Cardoso RA, et al. Clinical and laboratory features of Brazilian children with autoimmune hepatitis types 1 and 2. J Pediatr Gastroenterol Nutr 2000; 31(2): 4.
31. Najafi M, Kianifar HR, Mir-Nasseri SMM, et al. Autoimmune hepatitis in children, clinical features and biochemical of Iranian children. Govaresh 2005; 10: 103-7.
32. Gregorio GV, Portmann B, Mowat AP, Vergani D, Mieli-Vergani G. A 12- year-old girl with antimitochondrial antibody positive autoimmune hepatitis. J Hepatol 1997; 27: 751-4.
33. Kenny RP, Czaja AJ, Ludwig J, Dickson ER. Frequency and significance of antimitocondrial antibody in severe chronic active hepatitis. Dig Dis Sci 1986; 31: 705-11.
34. Özsoylu S, Yüce A, Koçak N. Çocuklarda kronik aktif hepatitler: 47 vakanın analizi. (Chronic active hepatitis in children: analysis of 47 cases). Turk J Gastroenterohepatol 1993; 4: 33-6.
35. Vento S, Garofano T, Di Perri G, Dolci L, Concia E, Bassetti D. Identification of hepatitis A virus as a trigger for autoimmune chronic hepatitis type 1 in susceptible individuals. Lancet 1991; 337: 1183-7.
36. Urganci N, Akyildiz B, Yildirmak Y, Ozbay G. A case of autoimmune hepatitis and autoimmune hemolytic anemia following hepatitis A infection. Turk J Gastroenterol 2003; 14(3): 204-7.
37. Pando M, Larriba J, Fernandez GC, et al. Pediatric and adult forms of type I autoimmune hepatitis in Argentina: evidence for differential genetic predisposition. Hepatology 1999; 30: 1374-80.
38. Duarte-Rey C, Pardo AL, Rodríguez-Velosa Y, Mantilla RD, Anaya JM, Rojas-Villarraga A. HLA class II association with autoimmune hepatitis in Latin America: a meta-analysis. Autoimmun Rev 2009; 8: 325-31.
39. Teufel A, Wörns M, Weinmann A, et al. Genetic association of autoimmune hepatitis and human leucocyte antigen in German patients. World J Gastroenterol 2006; 12: 5513-6.
40. Muratori P, Muratori L, Pappas G, et al. Genetic distinctions between autoimmune hepatitis in Italy and North America. World J Gastroenterol 2005; 11: 1862-6.
41. Umemura T, Ota M, Yoshizawa K, et al. Lack of association between FCRL3 and FcgammaRII polymorphisms in Japanese type 1 autoimmune hepatitis. Clin Immunol 2007; 122: 338-42.
42. Yokosawa S, Yoshizawa K, Ota M, et al. A genomewide DNA microsatellite association study of Japanese patients with autoimmune hepatitis type1. Hepatology 2007; 45: 384-90.
43. Yoshizawa K, Ota M, Katsuyama Y, et al. Genetic analysis of the HLA region of Japanese patients with type 1 autoimmune hepatitis. J Hepatol 2005; 42: 578-84.
44. Ma X, Qiu DK. Relationship between autoimmune hepatitis and HLA- DR4 and DRβ allelic sequences in the third hypervariable region in Chinese. World J Gastroenterol 2001; 7: 718-21.
45. Qiu DK, Ma X. Relationship between type I autoimmune hepatitis and alleles of HLA-DRB1 in Chinese patients of Shanghai area. Zhonghua Gan Zang Bing Za Zhi 2002; 10: 347-9.
46. Qiu DK, Ma X. Relationship between human leukocyte antigen-drb1 and autoimmune hepatitis type I in Chinese patients. J Gastroenterol Hepatol 2003; 18: 63-7.
47. Czaja AJ, Strettell MD, Thomson LJ, et al. Associations between alleles of the major histocompatibility complex and type1 autoimmune hepatitis. Hepatology 1997; 25: 317-23.
48. Strettell MD, Donaldson PT, Thomson LJ, et al. Allelic basis for HLA- encoded susceptibility to type 1 autoimmune hepatitis. Gastroenterology 1997; 112: 2028-35.
49. Heneghan MA, McFarlane IG. Current and novel immunosuppressive therapy for autoimmune hepatitis. Hepatology 2002; 35: 7-13.
50. Roberts EA. Autoimmune hepatitis. Indian J Pediatr 1995; 62: 525-31.
51. Alvarez F, Ciocca M, Canero-Velasco C, et al. Short-term cyclosporine induces a remission of autoimmune hepatitis in children. J Hepatol 1999; 30: 222-7.
52. Bae SH, Kim JS, Kim DH. Deflazacort for type-1 autoimmune hepatitis in a Korean girl. J Korean Med Sci 2006; 21: 758-60.
53. Bernardez RJ, Mimoso CC, Moreno PA, et al. Deflazocort for long-term maintenance of remission in type 1 autoimmune hepatitis. Rev Esp Enferm Dig 1999; 91: 630-8.
54. Mieli-Vergani G, Vergani D. Autoimmune paediatric liver disease. World J Gastroenterol 2008; 14(21): 3360-7.