Case of autoimmune hepatitis with markedly enlarged hepatoduodenal ligament lymph nodes

Abstract

Autoimmune hepatitis (AIH) is a necroinflammatory liver disease of unknown etiology. The disease is characterized histologically by interface hepatitis, biochemically by increased aspartate aminotransferase and alanine aminotransferase levels, and serologically by increased autoantibodies and immunoglobulin G levels. Here we discuss AIH in a previously healthy 37-year-old male with highly elevated serum levels of soluble interleukin-2 receptor and markedly enlarged hepatoduodenal ligament lymph nodes (HLLNs, diameter, 50 mm). Based on these observations, the differential diagnoses were AIH, lymphoma, or Castleman's disease. Liver biopsy revealed the features of interface hepatitis without bridging fibrosis along with plasma cell infiltration which is the typical characteristics of acute AIH. Lymph node biopsy revealed lymphoid follicles with inflammatory lymphocytic infiltration; immunohistochemical examination excluded the presence of lymphoma cells. Thereafter, he was administered corticosteroid therapy: after 2 mo, the enlarged liver reached an almost normal size and the enlarged HLLNs reduced in size. We could not find AIH cases with such enlarged lymph nodes (diameter, 50 mm) in our literature review. Hence, we speculate that markedly enlarged lymph nodes observed in our patient may be caused by a highly activated, humoral immune response in AIH.

Keywords: Autoimmune hepatitis; Corticosteroid; Hepatoduodenal ligament lymph nodes; Hepatomegaly; Humoral immune response.

Figure 1

Imaging findings. A: Dynamic computed tomographic image (CT) of a transverse section; B: A coronal section revealing enlargement of the hepatoduodenal ligament lymph nodes (arrows); C: Ultrasonography of the abdomen revealing a large mass in the region of the hepatic portal vein (arrow); D: Positron emission tomography/CT using ¹⁸F-fluorodeoxyglucose revealing mild accumulation. The maximum standardized uptake value was 3.98. The arrow shows the hepatoduodenal ligament lymph nodes.

Figure 2

Liver biopsy. A: Liver biopsy specimen with hematoxylin and eosin staining (× 100 magnification) revealing the histopathological appearance of acute hepatitis. Interface hepatitis and plasmacytic infiltrates are present; B: This is the same image at × 400 magnification. P: Portal area; C: Central vein area.

Figure 3

Histological sections of hepatoduodenal ligament lymph nodes (× 200 magnification). A: The lymphoid follicles contain a reactive germinal center with hematoxylin and eosin staining. No evidences of granuloma or necrosis are visible; B: CD3 (T-cell marker) is positive in the interfollicular areas; C: CD10 (a marker of B-cell activation) is positive in the center of the follicles; D: CD20 (B-cell marker) is observed in the nodules; E: Kappa chain; F: Lambda chain. Neither the kappa nor the lambda chains predominate.

Figure 4

Clinical course. The patients showed high levels of alanine aminotransferase, alkaline phosphatase and total bilirubin. However, after the initial corticosteroid therapy, these levels improved gradually. ALT: Alanine aminotransferase; ALP: Alkaline phosphatase; T-Bil: Total bilirubin.

Figure 5

Computed tomography of the transverse section recorded 2 mo after starting corticosteroid. The hepatoduodenal ligament lymph nodes reduced in size.