scholarly journals Immunoglobulin G4–Related Sclerosing Cholangitis Mimicking Hilar Cholangiocarcinoma Diagnosed With Following Bile Duct Resection: Report of a Case

2015 ◽  
Vol 100 (3) ◽  
pp. 480-485 ◽  
Author(s):  
Atsushi Miki ◽  
Yasunaru Sakuma ◽  
Hideyuki Ohzawa ◽  
Yukihiro Sanada ◽  
Hideki Sasanuma ◽  
...  

We report a rare case of immunoglobulin G4 (IgG4)–related sclerosing cholangitis without other organ involvement. A 69-year-old-man was referred for the evaluation of jaundice. Computed tomography revealed thickening of the bile duct wall, compressing the right portal vein. Endoscopic retrograde cholangiopancreatography showed a lesion extending from the proximal confluence of the common bile duct to the left and right hepatic ducts. Intraductal ultrasonography showed a bile duct mass invading the portal vein. Hilar bile duct cancer was initially diagnosed and percutaneous transhepatic portal vein embolization was performed, preceding a planned right hepatectomy. Strictures persisted despite steroid therapy. Therefore, partial resection of the common bile duct following choledochojejunostomy was performed. Histologic examination showed diffuse and severe lymphoplasmacytic infiltration, and abundant plasma cells, which stained positive for anti-IgG4 antibody. The final diagnosis was IgG4 sclerosing cholangitis. Types 3 and 4 IgG4 sclerosing cholangitis remains a challenge to differentiate from cholangiocarcinoma. A histopathologic diagnosis obtained with a less invasive approach avoided unnecessary hepatectomy.

2020 ◽  
Vol 48 (10) ◽  
pp. 030006052095921
Author(s):  
Cheng Xu ◽  
Yongmei Han

Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a novel clinical disease that is characterized by elevated serum IgG4 concentrations and tumefaction or tissue infiltrated by IgG4+ plasma cells. The clinical manifestations of IgG4-RD depend on the type of tissues affected. IgG4-related sclerosing cholangitis is a type of IgG4-RD. We report a patient who initially visited a local hospital with a 5-month history of jaundice. He was found to have a mass in the upper part of the common bile duct that mimicked cholangiocarcinoma. He underwent surgery in our hospital and was later diagnosed with IgG4-related sclerosing cholangitis. We administered prednisolone 40 mg once a day for treatment. Taking into account the possible side effects of moderate-dose hormone therapy, we also administered teprenone, potassium chloride, and calcium carbonate. The patient did not have any recurrence of symptoms or adverse drug reactions during follow-up.


1972 ◽  
Vol 25 (1) ◽  
pp. 155 ◽  
Author(s):  
I Caple ◽  
T Heath

Bile and pancreatic juice were collected from conscious, standing sheep with fistulae of the common bile duct, before and during infusions of secretin to the portal vein, and during infusion of acid to the duodenum. The output of volume and electrolytes, particularly bicarbonate, in bile and in pancreatic juice increased during infusion of secretin. However, the output of volume and of bicarbonate was three to five times higher in bile than in pancreatic juice. When acid was infused into the duodenum a similar result was obtained, and the increment in total bicarbonate output was similar to the amount of acid infused.


Author(s):  
A. E. Lychkova ◽  
A. Z. Tagirova ◽  
A. M. Puzikov

The aim—to characterize violations of the motor function of the biliary system in PSC in the clinic and experiment; to reveal the morphological correspondence of the studied pathology model.Materials and methods. Under observation were 20 patients with PSC in whom the motor function of the biliary system in the clinic was studied electromyographically. The frequency and amplitude of slow waves and spikes, the power of phase and tonic contractions were recorded on the EMG curve. PLC was simulated in 28 rats when 0.1 ml of picrylsulfonic acid was retrogradely injected into the common bile duct.Results. Electromyography of the common bile duct revealed an increase in the tone of circular muscles, a proportional increase in the power of phase and tonic contractions of the gallbladder with a decrease in the amplitude characteristics of spike activity. When simulating PSC, inhibition of EMG of the common bile duct and the detection of fibrosis of the type “bulbous husk” were noted.Conclusions. A decrease in the propulsive activity of the common bile duct in patients was found. The PSC model corresponds to the characteristics of this disease in the clinic.


Rangifer ◽  
1990 ◽  
Vol 10 (1) ◽  
pp. 17
Author(s):  
Timo Rahko ◽  
Sven Nikander

<p>In a previous publication the authors have described some ultrastructural characteristics of granulated cells in the common bile duct of the reindeer. On the basis of the same material, electron microscopic observations on other tissue elements of bile duct wall are now reported. The surface and glandular epithelium were composed of tall columnar epithelial cells with villous structures on the luminal surfaces. The parietal cytoplasmic membranes of epithelial cells were equipped with intercellular desmosomes while intraepithelial globule leucocytes did not form any junctional complex with other cells. Apical cytoplasmic areas of superficial epithelial cells showed electron-dense small bodies possibly consisting of mucinous substances. The goblet and deep glandular cells, on the other hand, contained numerous large mucin granules with less electron-dense matrices. It appears that their secretions are more abundant than those in superficial epithelial cells which obviously are absorptive as their main function. The nuclei and other cytoplasmic organelles showed profiles similar to those in epithelial cells generally. The lumen of the bile ducts was usually empty or contained fine-granular or amorphous material. An unusual feature was the presence of parts of globule leucocytes or even almost whole cells occurring freely in ductal secretions.</p><p>Elektronimikroskooppinen tutkimus yhteisen sappik&auml;yt&auml;v&auml;n rakenteesta porolla.</p><p>Abstract in Finnish / Yhteenveto: Aikaisemmassa julkaisussa tekij&auml;t kuvasivat poron yhteisen sappik&auml;yt&auml;v&auml;n (<em>ductus hepaticus communis</em>) sein&auml;m&auml;n jyv&auml;sellisten solujen hienorakennetta. T&auml;ss&auml; artikkelissa selostetaan saman aineiston perusteella (6 tervett&auml; teurasporoa) elektronimikroskooppisia havaintoja sappik&auml;yt&auml;v&auml;sein&auml;m&auml;n muista kudosrakenteista. Sappik&auml;yt&auml;v&auml;sein&auml;m&auml;n pinta- ja rauhasepiteeli koostuu korkeista epiteelisoluista. Pinnallisia epiteelisoluja kattavat s&auml;&auml;nn&ouml;lliset mikrovillukset, ja niill&auml; on vain v&auml;h&auml;n ilmeisesti limaa sis&auml;lt&auml;vi&auml; jyv&auml;si&auml; solulimassaan. Rau-has- ja pikarisoluissa s&auml;&auml;nn&ouml;lliset mikrovillukset sen sijaan puuttuvat. Niiden sytoplasman t&auml;ytt&auml;v&auml;t runsaat li-mapalloset, joita solut muodostavat hyvin kehittyneess&auml; Golgin laitteessaan eritt&auml;en limaa sappik&auml;yt&auml;v&auml;n onte-loon. Erite n&auml;kyy hienojyv&auml;isen&auml; tai tasa-aineisena sein&auml;mi&auml; reunustavana aineena. Poikkeuksellisena havaintona voidaan pit&auml;&auml; ker&auml;ssolujen ker&auml;sten tai l&auml;hes kokonaisten ker&auml;ssolujen esiintymist&auml; sappik&auml;yt&auml;v&auml;on-teloissa mahdollisesti osoituksena solujen vaelluskyvyst&auml;. Vaikka epiteelisolut muodostavat lujia solukalvosi-doksia toisiinsa, ker&auml;ssolut eiv&auml;t kiinnittyneet muihin soluihin. Ilmeist&auml; on, ett&auml; pinnallisten epiteelisolujen toiminta on p&auml;&auml;asiassa absorptiivista, mutta rauhasepiteelisolut ovat erikoistuneet eritystoimintaan.</p><p>En elektronmikroskopisk studie av gallg&aring;ngen hos ren.</p><p>Abstract in Swedish / Sammandrag: Gallg&aring;ngarnas yt- och k&ouml;rtelepitel bestod av h&ouml;ga epitelceller bekl&auml;dda med ett regelbundet villusskikt. Intercellulara desmosomer s&aring;gs i epitelcellernas parietala cytoplasmamembraner. De intraepiteliala globulara leukocyterna saknade desmosomer eller andra bindningar med n&aring;rliggande celler. I de superficiala epitelcellernas apikala cytoplasma fanns elektrontata sm&aring; kroppar antagligen best&aring;ende av mucin. B&auml;gar- och de djupare belagna k&ouml;rtelcellerna inneholl rikligt med stora mucin granulor med ett mindre elektrontatt matrix. Det f&ouml;ref&ouml;ll som om dessa celler skulle vara sekrerande och de superficiala epitelcellerna absorberande. K&auml;rnen och andra cytoplasmatiska organeller hade egenskaper jamf&ouml;rbara med epitelceller i allm&auml;nhet. Gallg&aring;ngen var oftast tom men ibland s&aring;gs ett finkornigt amorft material i den. Som en anmarkningsvard observation ansees forekomsten av delar och t.o.m. hela globulara leukocyter i gallg&aring;ngen.</p>


Kanzo ◽  
1991 ◽  
Vol 32 (8) ◽  
pp. 787-792
Author(s):  
Yukiyo SAKURADA ◽  
Toshihiro HISADOME ◽  
Norikuni ISHIMI ◽  
Toshio MURAKI ◽  
Satoshi HOSHIYA ◽  
...  

2012 ◽  
Vol 255 (3) ◽  
pp. 523-527 ◽  
Author(s):  
Juliette C. Slieker ◽  
Waqar R. R. Farid ◽  
Casper H. J. van Eijck ◽  
Johan F. Lange ◽  
Jasper van Bommel ◽  
...  

Author(s):  
Veeraraghavan Gunasekaran ◽  
Supraja Laguduva Mohan ◽  
Sunitha Vellathussery Chakkalakkoombil ◽  
Kuppusamy Senthamizhselvan

AbstractPortal cavernoma cholangiopathy (PCC) refers to the cholangiographic abnormalities that occur in patients with portal cavernoma. These abnormalities may be either due to chronic portal vein thrombosis or extrahepatic portal vein occlusion. These abnormalities occur due to enlargement of the bridging tortuous paracholedochal, epicholedochal, and cholecystic veins exerting pressure on the bile ducts. Ischemic changes can also occur in the bile duct due to portal vein thrombosis, which affects the microvascular circulation or associated hepatic artery thrombosis. These may be either reversible with shunt procedures such as transjugular intrahepatic portosystemic shunt (TIPS) or irreversible in the advanced stage, leading to recurrent episodes of biliary pain, cholangitis, and cholestasis. Occasionally it may present as mass-like diffuse common bile duct (CBD) wall thickening, which may be confused with mimickers like primary CBD lymphoma, immunoglobulin G4-related sclerosing cholangitis, and even cholangiocarcinoma on imaging. Thus, we need to be aware of the mass-forming PCC imaging findings to avoid unnecessary invasive procedures like biopsy or surgical intervention. Here, we present a case of PCC, which presented as mass-like diffuse CBD wall thickening with patent lumen on ultrasound that led to further workup with contrast-enhanced computed tomography and magnetic resonance imaging. The wall thickening showed persistent delayed enhancement, no significant diffusion restriction, and there was also associated superior mesenteric vein thrombosis with multiple mesenteric collaterals. A positron emission tomography-CT scan also ruled out malignant disease as there was no uptake. Finally, a diagnosis of mass-forming PCC was made by combining imaging features and excluding other causes.


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