CASE PRESENTATION: We report the case of a 56-year-old man who presented with obstructive jaundice with preoperative imaging showing proximal common bile duct obstruction. [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]

  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]

  Common initial symptoms include: Fatigue Itching of the skin Jaundice Diarrhea Weight loss / poor growth In more advanced disease, if the bile ducts become infected, patients may experience fever, chills and abdominal pain (cholangitis). [cincinnatichildrens.org]

• 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]

• Lymphedema

  A 52-year old patient presented with lymphedema, protein loosing enteropathy, and sclerosing cholangitis and was diagnosed with lymphedema cholestasis syndrome (LCS). [ncbi.nlm.nih.gov]

• 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]

• Pruritus

  Later in the course of the disease, patients present with symptoms of cholestasis (e.g., 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]

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

  Cholangiolocellular carcinoma is a rare malignant liver tumor that has been found in adults with chronic liver disease. We present a case of cholangiolocarcinoma found in an adolescent boy with small duct sclerosing cholangitis. [ncbi.nlm.nih.gov]

The dose and duration of glucocorticoid for treatment should be adjusted according to clinical situations, and proper maintaining dose is essential for a better prognosis. [ncbi.nlm.nih.gov]

Treatment Options There is currently no drug therapy approved for treatment of PSC in Germany. Classic anti-inflammatory drugs have been shown to be ineffective in various trials. [drfalkpharma.de]

Treatment for sclerosing cholangitis focuses on managing the disease and the symptoms it causes. Non-surgical treatments may include antihistamines and antibiotics. [stlouischildrens.org]

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

Prognosis of Primary Sclerosing Cholangitis Average duration to fulminant liver failure can range between 9 and 12 years. Several features correlate with a poor prognosis. [healthengine.com.au]

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]

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]

[…] 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]

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]

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]

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

[…] 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]

Treatment Symptomatic Ursodeoxycholic acid and immunosuppressives (e.g., tacrolimus ) : may decrease transaminases, ALP, and serum bilirubin, but do not prevent disease progression Treatment of pruritus (e.g., cholestyramine, rifampicin, naltrexone ) [amboss.com]