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

• Kang MK
• Park JG
• Choi JH

• KMID: 2501422
• DOI: http://doi.org/10.12701/yujm.2020.00066

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

References

1. Krawitt EL. Autoimmune hepatitis. N Engl J Med. 2006; 354:54–66.

2. Manns MP, Vogel A. Autoimmune hepatitis, from mechanisms to therapy. Hepatology. 2006; 43(2 Suppl 1):S132–44.

3. Vergani D, Longhi MS, Bogdanos DP, Ma Y, Mieli-Vergani G. Autoimmune hepatitis. Semin Immunopathol. 2009; 31:421–35.

4. Alvarez F, Berg PA, Bianchi FB, Bianchi L, Burroughs AK, Cancado EL, et al. International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol. 1999; 31:929–38.

5. Umemura T, Zen Y, Hamano H, Ichijo T, Kawa S, Nakanuma Y, et al. IgG4 associated autoimmune hepatitis: a differential diagnosis for classical autoimmune hepatitis. Gut. 2007; 56:1471–2.

6. Umemura T, Zen Y, Hamano H, Joshita S, Ichijo T, Yoshizawa K, et al. Clinical significance of immunoglobulin G4-associated autoimmune hepatitis. J Gastroenterol. 2011; 46(Suppl 1):48–55.

7. Rajkumar SV. Multiple myeloma: 2018 update on diagnosis, risk‐stratification, and management. Am J Hematol. 2018; 93:981–1114.

8. Canivet CM, Anty R, Patouraux S, Saint-Paul MC, Lebeaupin C, Gual P, et al. Immunoglobulin G4-associated autoimmune hepatitis may be found in Western countries. Dig Liver Dis. 2016; 48:302–8.

9. Amarapurkar AD, Amarapurkar DN. Immunoglobulin IgG4 and autoimmune hepatitis. Trop Gastroenterol. 2015; 36:112–7.

10. Chung H, Watanabe T, Kudo M, Maenishi O, Wakatsuki Y, Chiba T. Identification and characterization of IgG4-associated autoimmune hepatitis. Liver Int. 2010; 30:222–31.

11. Aydemir Y, Akcoren Z, Demir H, Saltik Temizel IN, Ozen H, Yuce A. Clinical and histopathological features of immunoglobulin G4-associated autoimmune hepatitis in children. J Gastroenterol Hepatol. 2019; 34:742–6.

12. Czaja AJ. Autoantibody-negative autoimmune hepatitis. Dig Dis Sci. 2012; 57:610–24.

13. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012; 366:539–51.

14. Watanabe T, Minaga K, Kamata K, Kudo M, Strober W. Mechanistic insights into autoimmune pancreatitis and IgG4-related disease. Trends Immunol. 2018; 39:874–89.

15. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: autoimmune hepatitis. J Hepatol. 2015; 63:971–1004.

16. Minaga K, Watanabe T, Chung H, Kudo M. Autoimmune hepatitis and IgG4-related disease. World J Gastroenterol. 2019; 25:2308–14.