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• W4313397244 abstract "View Large Image Figure ViewerDownload Hi-res image Download (PPT) Question: A 53-year-old man was admitted to our hospital owing to a 2-month history of right upper quadrant abdominal pain with no weight loss. He had no history of drinking or any other documented medication, and denied fever. He never ate raw or undercooked meat and seafood. Two large hepatic masses were detected by screening ultrasound. Abnormalities were not shown in the extrahepatic bile ducts and pancreas by imaging analyses. White blood cell and eosinophil counts were normal. The hemoglobin level was 12.0 g/dL (normal range 13–17.5 g/dL). Aspartate transaminase and alanine transaminase were normal. Alkaline phosphatase (ALP) was 239 IU/L (normal range 50–130 IU/L), and gamma-glutamyl transpeptidase (γ-GTP) was 171 IU/L (normal range 10–60 IU/L). He had a low serum albumin level (35 g/L). Laboratory examination results suggestive of infection with hepatitis A, B, C, and E were negative. Serum alpha-fetoprotein was within the normal range. Initial contrast-enhanced abdominal computed tomography (CT) was performed owing to suspicion of a malignant hepatic tumor. CT confirmed 2 masses 40 mm and 45 mm in diameter with characteristics of a peripheral rim of heterogeneous enhancement in the right hepatic lobe, which resembled the bull’s-eye sign (Figure A). The bull’s-eye sign was more prominent in conventional portal venous-phase contrast-enhanced CT imaging compared with arterial-phase (Figure B). Chest CT, esophagogastroduodenoscopy and colonoscopy were normal. Screening was also negative for the following autoantibodies: antinuclear antibody, anti–double-stranded DNA, antimitochondrial antibodies, anti–liver/kidney microsomal type 1, anti–smooth muscle antibody, anti–SSA/Ro, anti–SSB/La, anti–Scl-70, cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA), and perinuclear antineutrophil cytoplasmic antibody (p-ANCA). CT guided core needle biopsy of the hepatic lesions was done to get adequate tissue for confirmative diagnosis. Photomicrographs of the histologic liver specimen revealed inflammatory cell infiltration consisting of lymphocytes, plasma cells, and neutrophils (Figure C). What else will you do to establish the diagnosis? Look on page 1057 for the answer and see the Gastroenterology website (www.gastrojournal.org) for more information on submitting to Gastro Curbside Consult. Clinically, abnormal hepatic imaging findings or lesions are common. A wide differential diagnosis should be considered, including primary hepatocellular carcinoma, liver metastases, intrahepatic cholangiocarcinoma, liver abscesses, primary hepatic lymphoma, inflammatory myofibroblastic tumor, hepatic parasites, and lymphoproliferative disorders. The bull’s-eye sign is a typical imaging manifestation of metastatic liver cancer, which is most common in liver metastasis of extrahepatic adenocarcinoma. Histopathologically, the central low-density area represents liquefied necrotic tissue, the peripheral rim of high density is filled with the tumor tissue, and the outer low-density area is the compressed normal liver tissue. However, We did not find any evidence of extrahepatic tumors. The above-mentioned common hepatic lesions were excluded from this case by histopathology. The pathologists came up with the possibility of IgG4-related disease (IgG4-RD). IgG4 staining was carried out, considering plenty of lymphocyte and plasma cell infiltration. Surprisingly, immunohistochemistry convincingly demonstrated enriched IgG4 lymphoplasmacytic infiltration (IgG4:IgG ratio >0.4), and a tremendous amount of IgG4-positive cells (>80 per high-power field) (Figure D), which was consistent with IgG4-RD. Subsequently, laboratory examination revealed that the patient’s serum IgG4 concentration was elevated to 10 times the upper limit of normal. He was free of swollen lachrymal and salivary glands and of lymphadenopathy. The final diagnosis was definite hepatic involvement in IgG4-related disease according to histopathologic and serologic analyses. IgG4-RD is an emerging systemic disease entity, most commonly affecting middle-aged to elderly men, that initially presents as an inflammatory process and then progresses to fibrosis and sclerosis.1Stone J.H. Zen Y. Deshpande V. IgG4-related disease.N Engl J Med. 2012; 366: 539-551Crossref PubMed Scopus (1931) Google Scholar IgG4-RD commonly involves pancreas, hepatobiliary tract, retroperitoneal fibrosis, lymph node, pulmonary, and kidney.2Cai Z-P, Fan F-C, Liu W. Vague upper abdominal pain—IgG4-related pancreatitis. Indian J Surg. Published online November 1, 2022. https://doi.org/10.1007/s12262-022-03594-yGoogle Scholar It is necessary to establish an interdisciplinary approach integrating histopathology and imaging for diagnosis. Classic histopathologic characteristics include IgG4-enriched lymphoplasmacytic infiltration (IgG4:IgG ratio >0.4), storiform fibrosis, and obliterative phlebitis, which currently represents the single best criterion standard for diagnosis.3Löhr J.M. Vujasinovic M. Rosendahl J. et al.IgG4-related diseases of the digestive tract.Nat Rev Gastroenterol Hepatol. 2022; 19: 185-197Crossref PubMed Scopus (13) Google Scholar IgG4-related hepatopathy is usually referred to as parenchymal involvement of IgG4-RD of the liver, whose pathogenesis and imaging findings are rarely described.4Kim Y.R. Lee Y.H. Choi K.H. et al.Imaging findings of IgG4-related hepatopathy: a rare case presenting as a hepatic mass.Clin Imaging. 2018; 51: 248-251Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar Histopathologically, IgG4-related hepatopathy includes heterogeneous histologic lesions in liver parenchyma.5Lee H.E. Zhang L. Immunoglobulin G4-related hepatobiliary disease.Semin Diagn Pathol. 2019; 36: 423-433Crossref PubMed Scopus (12) Google Scholar However, IgG4-related hepatopathy with an infiltrative enhancing mass is considered to be an atypical and protean form, which is easily misdiagnosed as a malignant hepatic tumor.6Chen J.H. Deshpande V. IgG4-related disease and the liver.Gastroenterol Clin North Am. 2017; 46: 195-216Abstract Full Text Full Text PDF PubMed Scopus (23) Google Scholar Futile and potentially harmful surgical procedures may be avoided if IgG4-hepatopathy attracts considerable attention in the differential diagnosis. Almost all hepatic IgG4-RD lesions are not easy to be confirmed owing to rarity and confusion of the multi-organ immune-mediated disease. As seen in the present patient, minimally invasive biopsy by needle puncture was usually required for the final diagnosis. Substantial amounts of IgG4-bearing cells in IgG4-immunostained liver biopsy contribute to the final diagnosis of IgG4-hepatopathy. Although IgG4-RD responds rapidly to immunosuppressive therapy, a large number of patients relapse after withdrawal of immunosuppression. Therefore, patients are usually placed on low-dose corticosteroid and maintenance therapy to prevent flares, which is especially used in Asia.7Yamamoto M. Takahashi H. Shinomura Y. Mechanisms and assessment of IgG4-related disease: lessons for the rheumatologist.Nat Rev Rheumatol. 2014; 10: 148-159Crossref PubMed Scopus (141) Google Scholar Azathioprine, methotrexate, and mycophenolate mofetil are also used frequently in IgG4-RD, aiming to achieve additional immunosuppression and spare patients the effects of long-term glucocorticoids.8Kamisawa T. Zen Y. Pillai S. et al.IgG4-related disease.Lancet. 2015; 385: 1460-1471Abstract Full Text Full Text PDF PubMed Scopus (817) Google Scholar The patient was initially treated with prednisolone at a dose of 0.5 mg/kg body weight per day for 1 month, which was tapered over a period of 6 months to 5.0 mg/d. Then methotrexate 10 mg/wk was begun. Clinical and serologic improvement after the start of glucocorticoid therapy was rapid for the patient. He received an outpatient follow-up serologic assessment 1 month after treatment initiation. The levels of hemoglobin, ALP, γ-GTP, serum albumin, and serum IgG4 concentration returned to normal half a year later (Figure E). Follow-up radiologic assessment begun 2 months later. After symptomatic therapy for a year, repeated CT showed complete disappearance of the hepatic lesions (Figure F)." @default.
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• W4313397244 date "2023-06-01" @default.
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• W4313397244 title "The Bull’s-Eye Sign in Liver: Are They Liver Metastases?" @default.
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