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Case Reports
. 2018 Sep 16;2018:2309293.
doi: 10.1155/2018/2309293. eCollection 2018.

IgG4-Related Sclerosing Cholangitis Involving the Intrahepatic Bile Ducts Diagnosed With Liver Biopsy

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Case Reports

IgG4-Related Sclerosing Cholangitis Involving the Intrahepatic Bile Ducts Diagnosed With Liver Biopsy

Malene Theilmann Thinesen et al. Case Rep Pathol. .
Free PMC article

Abstract

IgG4-related disease is characterized by lymphoplasmacytic inflammation and fibrosis, often leading to mass-forming lesions in different organs. When IgG4-related disease affects the bile ducts, it is called IgG4-related sclerosing cholangitis. A 74-year-old male complained of dysphagia and abdominal pain. Endoscopic retrograde cholangiography and magnetic resonance cholangiography revealed bile duct changes suspicious of a bile duct carcinoma or cholangitis. Liver biopsy showed storiform fibrosis, lymphoplasmacytic infiltration, obliterative phlebitis, and a portal-based inflammatory nodule with expansion of a portal tract. Hot spots revealed 339 IgG4-positive cells per high power field (HPF) and an IgG4/IgG ratio of 72%. Eight months earlier, an inguinal lymph node had been removed, showing expanded interfollicular zones and increased plasma cells. Hot spots revealed 593 IgG4-positive cells and an IgG4/IgG ratio of 92%. The serum IgG4 of the patient was elevated nearly 10 times upper limit of normal. The diagnosis of IgG4-related sclerosing cholangitis associated with IgG4-related lymphadenopathy was made. There was good response to treatment with prednisolone and azathioprine. The differentiation of IgG4-related sclerosing cholangitis from primary sclerosing cholangitis and bile duct carcinoma is often difficult. Liver biopsy only rarely contributes to this setting, but we describe and report in detail a case where liver biopsy showed a portal-based inflammatory nodule with the characteristic features of this disease.

Figures

Figure 1
Figure 1
Imaging findings in a patient with IgG4-related sclerosing cholangitis (IgG4-SC). (a) At magnetic resonance imaging, no tumor-like lesions are seen. A benign hepatic cyst is indicated (1). (b) Diffuse and multiple intrahepatic biliary stenoses and dilatations at endoscopic retrograde cholangiography (1). The stenoses are relatively long. In classical primary sclerosing cholangitis (PSC), the stenoses would have been shorter, giving the cholangiogram a more beaded appearance. No complete obstruction of a bile duct is seen, a finding that would have been more characteristic of bile duct carcinoma. 2: occlusive balloon, 3: endoscope, 4: guide wire.
Figure 2
Figure 2
Liver core needle biopsy findings in a patient with IgG4-related sclerosing cholangitis (IgG4-SC). (a) A portal inflammatory nodule (IN), showing storiform fibrosis (arrows) and diffuse lymphoplasmacytic infiltration (H&E). (b) Higher magnification of the storiform fibrosis (H&E). (c) Strong infiltration of lymphocytes and plasma cells in the wall of the bile duct (Methyl-Green Pyronin). (d) Deeper section of the same area shown in Figure 2(a). At the periphery of the IN, there is obliterative phlebitis (arrows). Note the wall of the corresponding artery (Verhoeff elastin). (e) Higher magnification of the area with obliterative phlebitis shown in Figure 2(d). The former lumen of the vein is filled with plasma cells and lymphocytes. The vein can only be identified using an elastin stain (Verhoeff elastin). (f) Deeper sections of the area shown in Figure 2(a). Strong infiltration of IgG4-positive cells in the IN. (g, h) The IgG4 / IgG ratio is 72%.
Figure 3
Figure 3
Histological findings in a patient with IgG4-related lymphadenopathy. (a) Low magnification showing expansion of the interfollicular zone (H&E). (b) At higher magnification, numerous plasma cells are identified in the interfollicular zones (H&E). (c, d) Strong infiltration of IgG4-positive cells, hot spot (c) (IgG4 immunostaining) and IgG-positive cells, hot spot (d) (IgG immunostaining). The IgG4 / IgG ratio is 92%. (e) A granulomatous focus, arranged in a ring-like fashion around a lymphoid follicle, is shown, a feature called perifollicular granuloma (H&E). (f) Another perifollicular granuloma, accompanied by numerous IgG4-positive cells (IgG4 immunostaining).

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