Acute-onset Autoimmune Hepatitis in a Patient with Selective Immunoglobulin M Deficiency

Abstract

Selective immunoglobulin M deficiency (SIGMD) is an uncommon primary immunodeficiency disorder. We herein report an SIGMD patient with autoimmune hepatitis. A 21-year-old Japanese man was transferred to our hospital because of acute liver dysfunction. His serum IgM level was low, whereas those of IgG and IgA were normal, indicating that he had SIGMD. We diagnosed him with acute-onset autoimmune hepatitis, and his liver function test findings gradually recovered with corticosteroid administration. Although SIGMD with autoimmune diseases has been reported, the clinical features and pathogenesis have not yet been clarified. We have summarized previous reports on SIGMD patients with autoimmune diseases.

Keywords: autoimmune hepatitis; selective immunoglobulin M deficiency.

Figure 1.

Abdominal contrast enhanced computed tomography scans. (A) Areas of low attenuation around the portal vein and its branches (periportal collar signs) were recognized. (B) Edematous thickening of the gallbladder wall appeared.

Figure 2.

An image of a liver biopsy specimen (Hematoxylin and Eosin staining, original magnification ×200). The presence of interface hepatitis with dense portal lymphocyte infiltrate disrupting the limiting plate is shown.

Figure 3.

The clinical course of the present case after admission to the previous hospital. ALT: alanine aminotransferase, AST: aspartate aminotransferase, IgG: Immunoglobulin G, UDCA: ursodeoxycholic acid