We present three cases preoperatively diagnosed and surgically treated as hilar cholangiocarcinoma. First and second cases presented with cholangiocarcinoma with portal vein involvement and third with a malignant-appearing hilar stricture. [ncbi.nlm.nih.gov]

• Fever

  A 63-year-old male was admitted to our hospital for frequent fever. Abdominal magnetic resonance cholangiopancreatography showed diffuse narrowing of the common bile duct and post-stenotic dilatation of the right posterior bile duct. [ncbi.nlm.nih.gov]

  […] disease The diagnosis of primary sclerosing cholangitis should be suspected in a patient with ulcerative colitis who has abnormal LFTs, especially a raised alkaline phosphatase pruritus and fatigue are early symptoms - patients may also present with fevers [gpnotebook.co.uk]

  Some patients present with pruritus (itching), jaundice (yellow skin caused by bilirubin retention), fatigue, fever, weight loss or signs of advanced liver disease. [texasliver.com]

  For those who do, symptoms include: fatigue (the most common symptom of PSC) itchy skin unexpected weight loss abdominal pain unexplained fever As the condition progresses, a person with PSC may develop symptoms of liver failure, including: jaundice pain [childrenshospital.org]

  As primary sclerosing cholangitis progresses, individuals typically develop right upper abdominal pain, fever, fatigue, pruritus, and jaundice. These individuals also are at risk of developing primary sclerosing cholangitis complications. [emedicinehealth.com]

• Surgical Procedure

  procedures may be done: Inserting a long, thin tube with a balloon at the end to open up narrowing ( endoscopic balloon dilation of strictures) Placement of a drain or tube for major narrowing (strictures) of bile ducts Proctocolectomy (removal of colon [medlineplus.gov]

  The risk of infection is particularly high after you've had a surgical procedure to expand a badly scarred bile duct or remove a stone blocking a bile duct. Portal hypertension. [mayoclinic.org]

  In some cases, endoscopic or surgical procedures may be used to open major blockages in bile ducts. Through an endoscope, the physician places a tiny tube with a balloon on the end into the narrowed bile duct. [gicare.com]

  This open surgical procedure is performed in the operating room under a general anesthetic. [virginiamason.org]

  The patient’s history included several surgical procedures: meniscus in 1985, thymoma with benign histology in 1999, nasal sinus polyps more than 20 years ago and again in 2008, varicose veins in 2006, cystocele in 2008, and hip fracture in July 2010. [revistanefrologia.com]

• Pseudotumor

  KEYWORDS: Autoimmune pancreatitis; IgG4; IgG4-related disease; Inflammatory pseudotumor; Sclerosing cholangitis [ncbi.nlm.nih.gov]

  Frequent findings include 2-4 : periductal fibrosis (onion skin lesions) periportal eosinophilic infiltrate paucity of ducts Associations ulcerative colitis Sjogren syndrome 6 retroperitoneal fibrosis 6 mediastinal fibrosis Riedel thyroiditis orbital pseudotumor [radiopaedia.org]

  Other associated conditions include cirrhosis, chronic active hepatitis, pericholangitis, pancreatitis, retroperitoneal or mediastinal fibrosis, Peyronie disease, Riedel thyroiditis, hypothyroidism, or retro-orbital pseudotumor. [sonoworld.com]

• Fishing

  The complementary use of FISH increased the sensitivity of hematoxylin-and-eosin (H&E) staining from 69.4% to 77.6%; the specificity was not reduced when either H&E or FISH was positive. [ncbi.nlm.nih.gov]

  Red arrows represent assumes stable findings; CA 19-9: Carbohydrate antigen 19-9; CCA: Cholangiocarcinoma; ERC: Endoscopic retrograde cholangiography; GB: Gallbladder; GBC: Gallbladder cancer; FISH: Fluorescence in situ hybridization; HCC: Hepatocellular [wjgnet.com]

  Biopsy can also be supplemented with a fluorescent-in-situ- hybridization (FISH) study to detect chromosome anomalies, if this technique is available ( 17 ). [aerzteblatt.de]

• Hodgkin Lymphoma

  Brain imaging showed a mass in the right frontal region, which on histopathology was shown to be high-grade non-Hodgkin lymphoma. Magnetic resonance cholangiopancreatography showed features of sclerosing cholangitis. [ncbi.nlm.nih.gov]

  This nonmetastatic cholestasis has been described in non-Hodgkin’s lymphoma, prostate cancer, and renal cell carcinoma. 35 - 38 Granulomatous Liver Diseases Granulomatous liver diseases are more accurately classified as infiltrative diseases but are discussed [clevelandclinicmeded.com]

• Recurrent Abdominal Pain

  Prednisolone was reduced to 5 mg every other day without consultation with his doctor 1 year ago in May 2017, then he presented to our hospital again with recurrent abdominal pain and jaundice. [ncbi.nlm.nih.gov]

• Severe Abdominal Pain

  However, 3 months after his first admission, he had more severe abdominal pain and further elevated serum CA19-9. [ncbi.nlm.nih.gov]

• Liver Mass

  Right upper quadrant ultrasound can be helpful to establish the presence of biliary dilatation or liver masses which can be associated with biliary obstruction and cholestatic liver chemistries. [cancertherapyadvisor.com]

• Pruritus

  pruritus, jaundice ). Laboratory abnormalities include elevated liver function tests and autoantibodies ( pANCA in up to 80% of cases). [amboss.com]

  Symptoms of PSC include intermittent malaise, pruritus, fever, chills, night sweats, and right upper abdominal quadrant pain. [e-guide.ecco-ibd.eu]

  Presentations of primary sclerosing cholangitis History: Fatigue Pruritus Weight loss Right upper quadrant (RUQ) pain Recurrent biliary infections Examination: Jaundice Hepatomegaly Features of chronic liver disease Dupuytren’s contracture; palmar erythema [oxfordmedicaleducation.com]

  The hallmark clinical feature of intrahepatic cholestasis of pregnancy is pruritus. [clevelandclinicmeded.com]

  […] be asymptomatic at the time of diagnosis, although they may have advanced disease The diagnosis of primary sclerosing cholangitis should be suspected in a patient with ulcerative colitis who has abnormal LFTs, especially a raised alkaline phosphatase pruritus [gpnotebook.co.uk]

• Eczema

  We report a male child with WAS, who had presented with recurrent pneumonia, eczema, thrombocytopenia, autoimmune hemolytic anemia, and vasculitic skin lesions. Genetic analysis revealed a classical genotype WAS 155C T; R41X. [ncbi.nlm.nih.gov]

• Erythema

  gynaecomastia; spider naevi; ascites; cachexia Features of IBD Stoma; abdominal scars; mouth ulcers; erythema nodosum Differential diagnosis of primary sclerosing cholangitis Any other cause of chronic liver disease (see chronic liver disease section [oxfordmedicaleducation.com]

• Withdrawn

  Prednisolone was started from 40 mg daily, tapered gradually, and totally withdrawn after 22 months of treatment. A new-onset cholangitis was detected 2 months later. Prednisolone 10 mg daily was administered again. [ncbi.nlm.nih.gov]

  The two patients that did not receive allocated treatment had withdrawn their consent. As expected, around 60%-70% of the patients in each group were male. [mdedge.com]

(PTC) Most invasive test For patients who cannot undergo ERCP Findings: multifocal strictures alternating with dilation and beading of bile ducts Ultrasound Following diagnosis colonoscopy (to assess for UC) Liver biopsy Not an essential part of the workup [amboss.com]

ERCP is a key step in the workup of primary sclerosing cholangitis and remains the gold standard for the diagnosis. [sonoworld.com]

Liver function tests are the most valuable in the laboratory workup, including levels of serum alkaline phosphatase, gamma glutamyl transpeptidase, and serum aminotransferase, and the presence of hypergammaglobulinemia. (See Workup .) [emedicine.medscape.com]

Of those patients, 43 (91.5%) had undergone liver biopsy; 33/43 patients (76.7%) had received cholangiography as part of their diagnostic workup. [scielo.br]

• Small Liver

  Several years after this diagnosis, a small liver lesion was found incidentally on computed tomography scan following a motorcycle accident. This lesion was shown to be stable by magnetic resonance imaging over the course of 2 years. [ncbi.nlm.nih.gov]

Treatment was replaced by mycophenolate mofetil. Although the relapse rate was reduced, the disease progressed to cirrhosis. [ncbi.nlm.nih.gov]

Treatment-emergent adverse events occurred in 80%, 59%, 73%, and 67%. [mdedge.com]

KEYWORDS: IgG4; IgG4-SC; PSC/AIH overlap; Prognosis [ncbi.nlm.nih.gov]

CMV has been implicated as a possible etiological agent in primary SC partly because of the ability to cause liver damage and its relationship with smooth muscle antibodies. [ncbi.nlm.nih.gov]

While the exact etiology is unknown, there is a strong association with autoimmune diseases, particularly ulcerative colitis (UC). In the early stages, PSC is usually asymptomatic. [amboss.com]

Etiology The precise etiology of PSC has not yet been established, but it is believed to be multifactorial. [orpha.net]

[…] liver injury, acute liver failure, biliary atresia, liver diseases in pregnancy, liver transplantation, gallstone disease, cholangiocarcinoma, acute and chronic pancreatitis, and pancreatic cancer, including information on pathogenesis, epidemiology, etiology [medical-dictionary.thefreedictionary.com]

Sponsor: NGM Biopharmaceuticals, Inc Eligibility Requirements: -Inclusion Criteria: Confirmed diagnosis of PSC -Exclusion Criteria: Clinically significant acute or chronic liver disease of an etiology other than PSC Secondary or IgG4 related sclerosing [medicine.vumc.org]

Epidemiology Sex : (2:1) Age : The median age at diagnosis is 40. References: [1] Epidemiological data refers to the US, unless otherwise specified. [amboss.com]

Links: aetiology epidemiology clinical features investigations treatment prognosis [gpnotebook.co.uk]

[…] drug-induced liver injury, acute liver failure, biliary atresia, liver diseases in pregnancy, liver transplantation, gallstone disease, cholangiocarcinoma, acute and chronic pancreatitis, and pancreatic cancer, including information on pathogenesis, epidemiology [medical-dictionary.thefreedictionary.com]

Cancer risk in primary sclerosing cholangitis: Epidemiology, prevention, and surveillance strategies. [wjgnet.com]

Summary Epidemiology The prevalence of PSC in Europe ranges from 1/446,000-1/6,170. A male preponderance is observed with a male to female ratio of approximately 2:1. [orpha.net]

This video covers the known pathophysiology, important signs and symptoms, diagnosis strategies, and treatment options for primary sclerosing cholangitis (PSC). [cincinnatichildrens.org]

Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology/Diagnosis/Management. 10th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 68. Zyromski NJ, Pitt HA. The management of primary sclerosing cholangitis. [medlineplus.gov]

Approximately 80% of patients with PSC have inflammatory bowel disease, more commonly ulcerative colitis than Crohn’s disease. 21 Pathophysiology and Natural History The pathophysiology of PSC is unclear, but there is evidence suggesting an autoimmune [clevelandclinicmeded.com]

Sleisenger and Fordtran's gastrointestinal and liver disease: pathophysiology, diagnosis, management (8th ed.). Philadelphia: Saunders. a b Williamson, KD; Chapman, RW (June 2015). "Primary sclerosing cholangitis: a clinical update". [en.wikipedia.org]

Pathogenesis Two major concepts are thought to be the underlying pathophysiological mechanism of SSC-CIP. The concept receiving the most attention is the so called “concept of ischemic cholangiopathy”. [dovepress.com]

Finally, using tacrolimus and mycofenolate mofetil as immunosuppressants to treat LCH might be favorable to prevent LCH recurrence. [ncbi.nlm.nih.gov]

Nevertheless, predictors of cancer in PSC are still largely unclear, preventive measures are for the most part unproven if not non-existent, and much is still unknown regarding prevention and surveillance of cancer in PSC[ 8 ]. [wjgnet.com]

[…] putative role of any particular pharmacologic agent in preventing cancer in patients with PSC. [gastroenterologyandhepatology.net]

See Answer Can primary sclerosing cholangitis be prevented? The specific cause of PSC is still unknown and therefore it seems not to be preventable. [medicinenet.com]

tacrolimus ) : may decrease transaminases, ALP, and serum bilirubin, but do not prevent disease progression Treatment of pruritus (e.g., cholestyramine, rifampicin, naltrexone ) Supplementation of fat-soluble vitamins (see “Complications” below) In [amboss.com]