J Korean Med Sci.  2006 Aug;21(4):758-760. 10.3346/jkms.2006.21.4.758.

Deflazacort for Type-1 Autoimmune Hepatitis in a Korean Girl

• Bae SH
• Kim JS
• Kim DH

• KMID: 2157835
• DOI: http://doi.org/10.3346/jkms.2006.21.4.758

Abstract

Prednisone or prednisolone are the mainstay drug treatments for autoimmune hepatitis in children. However, long-term use of corticosteroid is associated with the risk of steroid-induced toxicities, and this situation requires newer immuno-suppressive agents for the treatment of autoimmune hepatitis, especially in growing children. An 11-yr-old Korean girl with type-1 autoimmune hepatitis discontinued prednisolone due to toxicities, i.e., hirsutism, buffalo hump, and skin striae, and remained clinical and biochemical remission under replacement of deflazacort and ursodeoxycholic acid combination therapy. A follow-up liver biopsy after 19 months of deflazacort and ursodeoxycholic acid treatment showed histologic remission.

Keyword

Hepatitis, Autoimmune; deflazacort; Child

MeSH Terms

Ursodeoxycholic Acid/therapeutic use
Treatment Outcome
Pregnenediones/*therapeutic use
Korea
Immunosuppressive Agents/therapeutic use
Humans
Hepatitis, Autoimmune/*drug therapy
Female
Drug Therapy, Combination
Cholagogues and Choleretics/therapeutic use
Child

Figure

• 

  Fig. 1 The pre-treatment liver biopsy shows moderate-lobular inflammation and mild portal inflammation (H&E, ×100). The inset shows piecemeal necrosis and moderate fibrosis (H&E, ×400).

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  Fig. 2 Post-treatment liver biopsy shows no lobular activity, and no portal activity (H&E, ×100). The inset shows near-normal periportal inflammation and fibrosis in the portal area (H&E, ×200).

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  Fig. 3 Clinical course of the patient. The patient manifested fulminent hepatitis with ANA positivity and a normal IgG level. Initially, a conventional steroid (prednisolone) and ursodeoxycholic acid was tried. After the development of steroid toxicities, deflazacort (Calcort®) was substituted for prednisolone. The patient remained on clinical and biochemical remission, and ANA became negative. A follow-up liver biopsy was performed. T.B, total bilirubin; UDCA, ursodeoxycholic acid; ANA, antinuclear antibody.

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