Immunoglobulin G4-related Hepatopathy after COVID-19 Vaccination

Abstract

An 84-year-old woman with IgG4-related disease presented with jaundice and liver dysfunction after coronavirus disease 2019 (COVID-19) vaccination. Serum IgG4 levels were elevated. Diagnostic imaging showed no stenotic lesions in the bile ducts. A liver biopsy was performed because of the enlarged liver. Infiltration of IgG4-positive plasma cells, which accounted for approximately 74% of total plasma cells, was found in the portal area, but there was no evidence of periportal hepatitis, and inflammatory cell infiltration into the lobular space was minimal. IgG4-related hepatopathy was diagnosed. The patient achieved spontaneous remission with no treatment and only follow-up and remains under observation at the time of writing.

Keywords: COVID-19 vaccination; IgG4-related hepatopathy.

Figure 1.

Computed tomography. At 80 years old: (a) Arrow indicates the hilar lymph node. (d) Arrow indicates the nodule in the lung. (g) Arrow indicates a mildly enlarged submandibular gland. At the onset: (b) Arrow indicates the hilar lymph node. (e) Arrow indicates nodule reduction in the lung. (i) Arrow indicates a mildly enlarged submandibular gland. At one year after the onset: (c) Arrow indicates the hilar lymph node. (f) Arrow indicates the disappearance of the nodule in the lung. (k) Arrow indicates a mildly enlarged submandibular gland. PET-CT findings, at 80 years old: (h) Arrow indicates a mildly enlarged submandibular gland. At the onset: (j) Arrow indicates a mildly enlarged submandibular gland.

Figure 2.

A lip biopsy showed a positive IgG4 (b)-to-CD138 (a) ratio of >70% (original magnification ×400), a: Arrow indicates CD138-positive plasma cell. b: Arrow indicates IgG4-positive plasma cell.

Figure 3.

(a) Findings of four examinations: at 80 years old, 1 month before the onset, at the onset (age 84 years old), 3 months after the onset. (b) Clinical course: the date of COVID-19 vaccination (arrow) is indicated by 0 on the horizontal axis.

Figure 4.

Liver biopsy findings. (a) The portal area was enlarged and well demarcated. Hematoxylin and Eosin (H&E) staining; original magnification ×10. (b) The portal area was highly infiltrated by lymphocytes and plasma cells. H&E staining; original magnification ×40. (c) Immunostaining of immunoglobulin G4 (IgG4) showed positive cells (brown color, arrow) in the portal area. Original magnification ×20. (d) Immunostaining of CD38 showed positive cells (brown color, arrow). Original magnification ×20. (e) An overall poor inflammatory response was seen, with necrosis in the lobules. H&E staining; original magnification ×20. (f) Within the sinusoids, lymphocytes were increased, as were macrophages positive for periodic acid-Schiff with diastase (dPAS) stain (arrows indicate representative examples). dPAS stain; original magnification ×20.