Yeungnam Univ J Med.  2020 Apr;37(2):136-140. 10.12701/yujm.2020.00066.

Anti-nuclear antibody-negative immunoglobulin G4-associated autoimmune hepatitis mimicking lymphoproliferative disorders

Affiliations
  • 1Department of Internal Medicine, Yeungnam University College of Medicine, Daegu, Korea
  • 2Department of Pathology, Yeungnam University College of Medicine, Daegu, Korea

Abstract

Immunoglobulin G4 (IgG4)-associated autoimmune hepatitis (AIH) is a very rare subtype of autoimmune hepatitis and characterized by marked elevated serum IgG and hepatic infiltration of IgG4-expressing plasma cells. Pathologic confirmation of hepatic IgG4-expressing plasma cells is usually required for the final diagnosis of IgG4-associated AIH. Herein, we report the case of a 47-year-old female diagnosed with autoantibody-negative IgG4-associated AIH mimicking lymphoproliferative disorders.

Keyword

Autoimmune hepatitis; Immunoglobulin G; Immunoglobulin G4-related disease; Plasma cells; Prednisolone

Figure

  • Fig. 1. Abdominal sonography and computed tomography showing (A) increased echogenicity of the liver parenchyma with heterogeneous echotexture and (B) normal pancreas without significant evidence of autoimmune pancreatitis.

  • Fig. 2. Histologic findings of liver. (A) Moderate interface hepatitis with lymphocytes and plasma cells infiltration in the portal tract is present (hematoxylin-eosin stain, ×200). (B) Immunoglobulin G4-positive plasma cells are seen (immunohistochemical stain, ×400).

  • Fig. 3. The clinical course of the patient. AST, aspartate aminotransferase; ALT, alanine aminotransferase; GGT, gamma-glutamyl transferase; IgG, immunoglobulin G.


Reference

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