Immunosuppressant treatment for IgG4-related sclerosing cholangitis: A case report

Figure 1 Computed tomography results. A: Intrahepatic duct (IHD, arrows); B: Common bile duct (CBD) dilatation without a definite obstructive lesion (arrows). After 3 mo of high-dose glucocorticoid therapy; C: IHD dilatation with intraductal soft tissue attenuation had disappeared; D: CBD dilatation with mild luminal narrowing was reduced.

Figure 2 Histological assessment of biopsy specimen. A: Biopsy of the ampulla of Vater showed inflammatory cell infiltration and granulation tissue [hematoxylin and eosin (H&E) stain, × 100 magnification]; B: Many plasma cells were observed in the subepithelial stroma (H&E stain, × 400 magnification); C: Immunohistochemical staining showed a large number of IgG4 positive cells (brown, × 400 magnification).

Figure 3 The initial laboratory findings indicated obstructive-pattern jaundice. Bilirubinemia (8.6 mg/dL) and a high C reactive protein (CRP) level (1.81 mg/dL) were noted. We applied high-dose systemic glucocorticoid (GC) for 3 d (prednisolone 0.5 mg/kg/d). After GC pulse treatment, the bilirubinemia and elevated CRP level had improved so we decided to administer CYC pulse therapy (1000 mg/mo, 6 mo). The maintenance therapy was prednisolone (5 mg/d) and mycophenolate mofetil (1000 mg/d). CRP: C reactive protein; MMF: Mycophenolate mofetil.