Przewlekła stwardniająca cholangitis

Przewlekła stwardniająca cholangitis
Diagnostyka i diagnoza

Przewlekła stwardniająca cholangitis (PSC) to przewlekła choroba wątroby charakteryzująca się postępującym zapaleniem, włóknieniem i zwężeniem dróg żółciowych wewnątrz- i zewnątrzwątrobowych. Diagnostyka opiera się na utrzymującym się ponad 6 miesięcy cholestatycznym profilu enzymów wątrobowych, w tym podwyższonym poziomie fosfatazy alkalicznej (ALP) i gamma-glutamylotranspeptydazy (GGTP), umiarkowanym wzroście aminotransferaz (ALT, AST) oraz obecności charakterystycznych zmian cholangiograficznych widocznych w MRCP, które wykazuje czułość 86% i swoistość 94%. W diagnostyce różnicowej należy wykluczyć wtórne przyczyny stwardniającego zapalenia dróg żółciowych oraz pierwotne zapalenie dróg żółciowych (PBC), które cechuje obecność przeciwciał przeciwmitochondrialnych (AMA). PSC silnie koreluje z nieswoistymi zapaleniami jelit (IBD), występując u 70-80% pacjentów, co wymaga wykonania kolonoskopii z biopsją w celu wykrycia współistniejącej choroby jelit.

Diagnostyka przewlekłej stwardniającej cholangitis

Objawy kliniczne i wywiad
Badania laboratoryjne
Badania obrazowe
Biopsja wątroby
Kryteria diagnostyczne
Badania dodatkowe i ocena chorób współistniejących
Diagnostyka różnicowa
Ocena rokowania

Podsumowanie diagnostyki PSC

Kolejne rozdziały

Diagnostyka przewlekłej stwardniającej cholangitis

Przewlekła stwardniająca cholangitis (Primary Sclerosing Cholangitis, PSC) jest rzadką, przewlekłą chorobą wątroby charakteryzującą się postępującym zapaleniem, włóknieniem i zwężeniem dróg żółciowych zarówno wewnątrzwątrobowych, jak i zewnątrzwątrobowych. Diagnostyka PSC opiera się na kombinacji wyników badań laboratoryjnych, obrazowych oraz wykluczeniu wtórnych przyczyn stwardniającego zapalenia dróg żółciowych.¹²

Objawy kliniczne i wywiad

Wielu pacjentów z PSC nie wykazuje objawów w momencie rozpoznania, a choroba często wykrywana jest przypadkowo podczas rutynowych badań krwi wykonywanych z innych powodów lub podczas badań przesiewowych.³⁴ U niektórych pacjentów mogą wystąpić niespecyficzne objawy, takie jak zmęczenie, dyskomfort w jamie brzusznej i silny świąd (pruritus).⁵⁶

Podczas zbierania wywiadu lekarz powinien zwrócić szczególną uwagę na historię chorób zapalnych jelit (IBD), ponieważ PSC silnie koreluje z nieswoistymi zapaleniami jelit, szczególnie z wrzodziejącym zapaleniem jelita grubego.⁷⁸ U około 70-80% pacjentów z PSC występuje IBD, a ich obecność jest silnym wskaźnikiem do sprawdzenia pod kątem PSC.⁹

Badanie fizykalne może wykazać żółtaczkę, powiększenie wątroby i śledziony, szczególnie w zaawansowanych stadiach choroby.¹⁰¹¹

Badania laboratoryjne

Badania laboratoryjne są zwykle pierwszym krokiem w diagnostyce PSC. Najbardziej charakterystycznym wzorcem biochemicznym jest cholestatyczny profil wątrobowy, który obejmuje:¹²¹³

Podwyższony poziom fosfatazy alkalicznej (ALP) – najwcześniejszy i najbardziej charakterystyczny marker, który zazwyczaj utrzymuje się przez ponad 6 miesięcy¹⁴¹⁵
Podwyższony poziom gamma-glutamylotranspeptydazy (GGTP)¹⁶
Umiarkowanie podwyższone aminotransferazy (ALT, AST)¹⁷
Hiperbilirubinemia – występuje w zaawansowanych przypadkach lub przy obecności dominującego zwężenia¹⁸

Chociaż nie istnieją specyficzne markery serologiczne dla PSC, u wielu pacjentów można zaobserwować obecność przeciwciał autoimmunologicznych:¹⁹²⁰

Perinuklearne przeciwciała przeciwko cytoplazmie neutrofilów (p-ANCA) – obecne u około 80% pacjentów z PSC²¹²²
Przeciwciała przeciwjądrowe (ANA) i przeciwciała przeciwko mięśniom gładkim (ASMA) – występują u około 75% pacjentów²³
Podwyższony poziom immunoglobulin, szczególnie IgM – u około 50% pacjentów²⁴

Ważne jest również wykluczenie innych chorób cholestatycznych, w szczególności pierwotnego zapalenia dróg żółciowych (PBC). Przeciwciała przeciwmitochondrialne (AMA), które są charakterystyczne dla PBC, są zwykle nieobecne w PSC.²⁵

Badania obrazowe

Badania obrazowe odgrywają kluczową rolę w potwierdzeniu diagnozy PSC. Charakterystyczne cechy cholangiograficzne obejmują obecność wieloogniskowych zwężeń i rozszerzeń wewnątrzwątrobowych i/lub zewnątrzwątrobowych dróg żółciowych, tworzących obraz „koralików” lub „suchego drzewa”.²⁶²⁷

Cholangiopankreatografia rezonansu magnetycznego (MRCP)

MRCP jest obecnie preferowaną metodą diagnostyczną pierwszego wyboru w diagnostyce PSC ze względu na jej nieinwazyjny charakter oraz wysoką czułość i swoistość.²⁸²⁹ Metaanalizy wykazały, że MRCP ma czułość 86% i swoistość 94% w wykrywaniu PSC w porównaniu do ERCP.³⁰³¹

MRCP wykorzystuje rezonans magnetyczny do uzyskania szczegółowych obrazów dróg żółciowych i trzustkowych, pozwalając na ocenę zmian strukturalnych charakterystycznych dla PSC, bez konieczności stosowania inwazyjnych technik i ekspozycji na promieniowanie.³²³³

Endoskopowa cholangiopankreatografia wsteczna (ERCP)

Historycznie ERCP była uważana za złoty standard w diagnostyce PSC, jednak ze względu na inwazyjny charakter procedury i ryzyko powikłań (około 10% pacjentów z PSC wymaga hospitalizacji po ERCP), jej rola diagnostyczna została ograniczona.³⁴³⁵

Obecnie ERCP jest zalecana głównie w celach terapeutycznych, takich jak:³⁶³⁷

Pobieranie próbek patologicznych z podejrzanych zwężeń³⁸
Rozszerzanie dominujących zwężeń³⁹
Usuwanie złogów i osadów w drogach żółciowych⁴⁰

ERCP może być również rozważana w przypadkach, gdy wyniki MRCP są niejednoznaczne lub gdy istnieje potrzeba jednoczesnej interwencji terapeutycznej w drogach żółciowych.⁴¹

Inne metody obrazowania

Ultrasonografia jamy brzusznej jest często pierwszym badaniem obrazowym wykonanym u pacjentów z podejrzeniem choroby wątroby, jednak ma ograniczoną wartość w diagnostyce PSC. Może ujawnić poszerzenie dróg żółciowych oraz zmiany w wątrobie i śledzionie, ale nie jest specyficzna dla PSC.⁴²⁴³

Tomografia komputerowa (CT) może być pomocna w ocenie zmian strukturalnych wątroby i dróg żółciowych, szczególnie w wykrywaniu powikłań, takich jak rak dróg żółciowych (cholangiocarcinoma).⁴⁴

Biopsja wątroby

Biopsja wątroby nie jest rutynowo wykonywana w diagnostyce PSC, szczególnie gdy wyniki badań obrazowych są charakterystyczne.⁴⁵⁴⁶ Jest jednak wskazana w następujących przypadkach:⁴⁷⁴⁸

Podejrzenie PSC małych przewodów (gdy MRCP jest prawidłowe, ale kliniczny obraz sugeruje PSC)⁴⁹
Podejrzenie zespołu nakładania z autoimmunologicznym zapaleniem wątroby (AIH)⁵⁰
Podejrzenie IgG4-zależnego stwardniającego zapalenia dróg żółciowych⁵¹
Niejednoznaczne wyniki innych badań diagnostycznych⁵²

Najbardziej charakterystyczną cechą histologiczną PSC jest koncentryczne włóknienie okołoprzewodowe („onion-skin fibrosis”) oraz zapalenie włóknisto-obliteracyjne dróg żółciowych, jednak zmiany te są obserwowane w mniej niż 15% biopsji.⁵³⁵⁴

Kryteria diagnostyczne

PSC jest diagnozowany na podstawie kombinacji następujących kryteriów:⁵⁵⁵⁶

Podwyższony poziom fosfatazy alkalicznej utrzymujący się przez ponad 6 miesięcy⁵⁷
Charakterystyczne zmiany cholangiograficzne w badaniach obrazowych (MRCP lub ERCP)⁵⁸
Wykluczenie wtórnych przyczyn stwardniającego zapalenia dróg żółciowych⁵⁹

PSC małych przewodów jest rozpoznawany, gdy występują typowe zmiany histopatologiczne przy prawidłowym obrazie cholangiograficznym.⁶⁰⁶¹

Badania dodatkowe i ocena chorób współistniejących

Ze względu na silny związek PSC z chorobami zapalnymi jelit oraz zwiększone ryzyko nowotworów, zaleca się przeprowadzenie dodatkowych badań:⁶²⁶³

Kolonoskopia z pobraniem wycinków – zalecana u wszystkich pacjentów z nowo rozpoznanym PSC w celu wykrycia IBD, nawet przy braku objawów jelitowych⁶⁴⁶⁵
Regularne badania przesiewowe w kierunku raka dróg żółciowych (cholangiocarcinoma) i raka jelita grubego u pacjentów z współistniejącym IBD⁶⁶⁶⁷
Oznaczenie poziomu markera nowotworowego CA 19-9, którego wartość powyżej 130 U/ml u pacjentów objawowych ma 79% czułość i 98% swoistość w wykrywaniu cholangiocarcinoma⁶⁸
Ocena gęstości mineralnej kości ze względu na zwiększone ryzyko osteoporozy⁶⁹

Diagnostyka różnicowa

Przed postawieniem diagnozy PSC konieczne jest wykluczenie wtórnych przyczyn stwardniającego zapalenia dróg żółciowych, które mogą naśladować obraz PSC:⁷⁰⁷¹

Polekowe uszkodzenie dróg żółciowych⁷²
Choroby nowotworowe dróg żółciowych⁷³
IgG4-zależne stwardniające zapalenie dróg żółciowych⁷⁴
Zakażenia oportunistyczne dróg żółciowych (szczególnie u pacjentów z HIV)⁷⁵
Pierwotne zapalenie dróg żółciowych (PBC)⁷⁶
Wtórne zmiany związane z zabiegami chirurgicznymi na drogach żółciowych⁷⁷

Ocena rokowania

Profil biochemiczny pacjenta w momencie diagnozy może dostarczyć informacji na temat rokowania. Najczęściej stosowanym modelem prognostycznym jest zrewidowany model Mayo dla PSC, który uwzględnia wiek, poziom bilirubiny, albuminy, AST oraz historię krwawień z żylaków.⁷⁸⁷⁹

Podwyższony poziom immunoglobuliny G4 (IgG4) może korelować z cięższym przebiegiem choroby.⁸⁰ Dominujące zwężenia dróg żółciowych są również niekorzystnym czynnikiem prognostycznym i mogą wymagać interwencji endoskopowej.⁸¹

Podsumowanie diagnostyki PSC

Diagnostyka przewlekłej stwardniającej cholangitis wymaga kompleksowego podejścia, obejmującego:⁸²

Ocenę kliniczną objawów i czynników ryzyka, ze szczególnym uwzględnieniem współistnienia IBD
Badania laboratoryjne wykazujące cholestatyczny profil enzymów wątrobowych (podwyższony ALP, GGTP)
Badania obrazowe – MRCP jako metoda pierwszego wyboru, a ERCP głównie do celów terapeutycznych
Biopsję wątroby w wybranych przypadkach (PSC małych przewodów, zespół nakładania z AIH)
Kolonoskopię w celu wykluczenia współistniejącego IBD
Wykluczenie wtórnych przyczyn stwardniającego zapalenia dróg żółciowych

Wczesne rozpoznanie PSC jest istotne ze względu na możliwość wcześniejszego wdrożenia monitorowania pod kątem powikłań, takich jak rak dróg żółciowych i rak jelita grubego, szczególnie u pacjentów z współistniejącym IBD.⁸³⁸⁴

Kolejne rozdziały

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Materiały źródłowe

#1 Primary Sclerosing Cholangitis – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK537181/
Primary sclerosing cholangitis (PSC) is a chronic and progressive cholestatic liver disorder of unknown etiology. […] This activity reviews the etiology, pathogenesis, evaluation, and management of primary sclerosing cholangitis and explains the role of the interprofessional team in evaluating and treating patients with this condition. […] Assess how to evaluate for primary sclerosing cholangitis. […] The diagnosis of PSC is typically made with the demonstration of characteristic multiple and focal areas of stricturing and dilation of intrahepatic or extrahepatic bile ducts on cholangiography. […] Diagnostic criteria for the diagnosis of PSC include: An increased serum alkaline phosphatase level that persists for more than 6 months, Cholangiographic findings of bile-duct strictures detected using either MRCP or ERCP, Exclusion of causes of secondary sclerosing cholangitis. […] The key feature in histology is periductal or onion skin fibrosis. […] There is no established treatment for PSC yet. […] The treatment of PSC requires an interprofessional approach.
#2 Primary Sclerosing Cholangitis â PSC | Choose the Right Test
https://arupconsult.com/content/primary-sclerosing-cholangitis-psc
Primary sclerosing cholangitis (PSC) is a progressive autoimmune liver disease (ALD) that often leads to cirrhosis and may eventually require liver transplantation. […] Many patients are diagnosed incidentally due to abnormal liver biochemistry or after an IBD diagnosis and subsequent biochemical screening suggestive of PSC. […] Laboratory testing and liver biopsy may assist in diagnosis; however, there are no autoantibodies or liver biochemistry profiles that are specific to PSC. […] Magnetic resonance cholangiopancreatography (MRCP) is the primary method of diagnosis for PSC, although diagnosis also involves exclusion of other causes of liver disease (e.g., toxic or infectious etiologies). […] Primary sclerosing cholangitis (PSC) is a diagnosis of exclusion and thus should only be diagnosed when other potential toxic, infectious, or inflammatory causes of the characteristic bile duct injury pattern are not present.
#3 Primary Sclerosing Cholangitis: Symptoms, Treatment & Diagnosis
https://my.clevelandclinic.org/health/diseases/23569-primary-sclerosing-cholangitis
Primary sclerosing cholangitis is a progressive disease that damages your bile ducts and your liver. It causes chronic inflammation and scarring. Scar tissue eventually restricts the flow of bile through the ducts and stops the liver from functioning properly. […] PSC is often found by accident while testing for something else. Up to 50% of people may not have any symptoms at the time of diagnosis. Early signs of the disease may show up on a blood test or imaging test. Images of bile ducts with PSC show certain distinctive features. A blood test may show high levels of alkaline phosphatase or certain antibodies that indicate an immune response in your bile ducts. White blood cell elevation is usually a sign of infection in the liver. […] To confirm the disease, your healthcare provider may suggest a more specific test, such as a liver function test. These blood tests look for high levels of particular liver enzymes. High levels of alkaline phosphatase may indicate PSC.
#4 Primary sclerosing cholangitis – Symptoms and causes – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/primary-sclerosing-cholangitis/symptoms-causes/syc-20355797
Primary sclerosing cholangitis is often diagnosed before symptoms appear when a routine blood test or an X-ray taken for an unrelated condition shows liver abnormalities. […] If primary sclerosing cholangitis is diagnosed, it’s important to look for inflammatory bowel disease because there is a greater risk of colon cancer. […] Primary sclerosing cholangitis and inflammatory bowel disease don’t always appear at the same time, though. In some cases, primary sclerosing cholangitis is present for years before inflammatory bowel disease occurs. […] Your doctor may recommend testing for inflammatory bowel disease, even if you have no signs or symptoms, since the risk of colon cancer is elevated if you have both diseases.
#5 Diagnosis of Primary Sclerosing Cholangitis – NIDDK
https://www.niddk.nih.gov/health-information/liver-disease/primary-sclerosing-cholangitis/diagnosis
Doctors diagnose primary sclerosing cholangitis (PSC) based on medical and family history, a physical exam, and the results of medical tests. […] The doctor will ask about your symptoms. […] Your doctor will examine your body, including your abdomen. […] Liver tests can show abnormal levels of liver enzymes and other substances in your blood. Abnormal levels of certain liver enzymes may be a sign your liver or bile ducts are damaged. […] To diagnose PSC, doctors typically order a special imaging test to examine the bile ducts, such as magnetic resonance cholangiopancreatography (MRCP), which uses a magnetic resonance imaging (MRI) machine to create pictures of the bile ducts. MRCP is the most common test that doctors use to diagnose PSC. […] A liver biopsy is generally not needed to diagnose PSC. However, in some cases, doctors may order a liver biopsy to check for signs of other liver diseases, such as autoimmune hepatitis. […] For people who have PSC and haven’t already been diagnosed with IBD, doctors may recommend a colonoscopy to check for IBD.
#6 Primary sclerosing cholangitis – Rabiee – Translational Gastroenterology and Hepatology
https://tgh.amegroups.org/article/view/6491/html
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease that is characterized by intra- and/or extrahepatic bile duct injury. The clinical presentation of PSC correlates with the sequence of inflammatory bile duct destruction and fibrosis, which results in bile duct stricturing, cholestasis, and eventually biliary cirrhosis with end-stage liver disease and hepatic dysfunction. PSC is increasingly diagnosed early in the stage of the disease course, and, as a result, the majority of patients do not have any clinical symptoms at the time of diagnosis. In the majority of cases, the diagnosis of PSC is prompted by the finding of cholestasis at the time of routine health evaluation or screening of high-risk patients such as those with inflammatory bowel disease (IBD). […] Currently, no autoimmune antibodies are sufficiently specific in the diagnosis of PSC. The most commonly positive autoantibodies are the perinuclear antineutrophilic autoantibodies (pANCA), which are found in approximately 80% of patients but lack diagnostic specificity. The diagnostic modality of choice for the diagnosis of PSC is magnetic resonance cholangiopancreatography (MRCP), which has been shown in a meta-analysis to have a sensitivity of 86% and specificity of 94% when compared to endoscopic retrograde cholangiopancreatography (ERCP). The diagnosis of PSC is typically established in patients with chronic cholestasis when cholangiographic studies (MRCP, ERCP, or percutaneous transhepatic cholangiogram) show characteristic features of PSC, and secondary forms of sclerosing cholangitis are excluded.
#7 Primary sclerosing cholangitis – Symptoms and causes – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/primary-sclerosing-cholangitis/symptoms-causes/syc-20355797
Primary sclerosing cholangitis is often diagnosed before symptoms appear when a routine blood test or an X-ray taken for an unrelated condition shows liver abnormalities. […] If primary sclerosing cholangitis is diagnosed, it’s important to look for inflammatory bowel disease because there is a greater risk of colon cancer. […] Primary sclerosing cholangitis and inflammatory bowel disease don’t always appear at the same time, though. In some cases, primary sclerosing cholangitis is present for years before inflammatory bowel disease occurs. […] Your doctor may recommend testing for inflammatory bowel disease, even if you have no signs or symptoms, since the risk of colon cancer is elevated if you have both diseases.
#8 Primary Sclerosing Cholangitis (PSC) – British Liver Trust
https://britishlivertrust.org.uk/information-and-support/liver-conditions/primary-sclerosing-cholangitis/
Many people have no symptoms at first and the disease is only discovered because of abnormal results of routine blood tests in patients with ulcerative colitis or Crohns disease. […] PSC is diagnosed on the combination of symptoms, blood tests and a picture of the bile ducts, called a cholangiogram. A liver biopsy may be needed not only to confirm the diagnosis, but also to assess either how early or advanced the disease is. […] If your GP suspects you may have the condition you will be referred to a hospital specialist for tests, expert advice and treatment.
#9 Diagnosing PSC – PSC Support
https://pscsupport.org.uk/diagnosing-psc/
There is no single diagnostic blood test for PSC. […] The diagnosis often comes unexpectedly after your routine blood tests for IBD suggest bile duct injury. […] Where there is any doubt about your PSC diagnosis, you should ask to be referred to a hospital or doctor with experience or an interest in PSC. […] However, because liver blood tests look normal in some people with PSC, blood tests alone cannot be used to diagnose it or rule it out. […] Around 70%-80% of people with PSC have IBD, and its presence is a strong indicator to check for PSC. […] If your doctor suspects PSC, you will be asked to have a type of MRI scan called an MRCP scan. It is the gold standard and the test of choice for diagnosing PSC. […] Where the MRCP shows a typical appearance for PSC, a liver biopsy is rarely undertaken. […] Once PSC is diagnosed, your doctor will check blood tests to rule out IGg4-associated cholangitis and sometimes cholangiocarcinoma as these can mimic and/or complicate PSC.
#10 Primary Sclerosing Cholangitis (PSC) | Boston Children’s Hospital
https://www.childrenshospital.org/conditions/primary-sclerosing-cholangitis
How is primary sclerosing cholangitis diagnosed? The first steps in diagnosing PSC are a physical exam and medical history. During the physical exam, the doctor will look for look for signs of liver damage and bile duct infection. The doctor may ask if the child has had symptoms such as fatigue, itchiness, unexplained weight loss, or abdominal pain. […] Information about the childs and familys health history will help the doctor assess the likelihood that a child has PSC. This may include whether any family members have PSC and whether the child has had inflammatory bowel disease or autoimmune disease. […] If the results of the medical history and physical exam show that a child could have PSC, the following tests can help determine if the disease is present or not with more certainty:
#11 Primary Sclerosing Cholangitis (PSC) and Its Importance in Clinical Practice | MDedge
https://www.mdedge.com/gihepnews/article/271472/primary-sclerosing-cholangitis-psc-and-its-importance-clinical-practice
Primary sclerosing cholangitis (PSC) is a rare, chronic, and progressive cholestatic liver disorder. […] PSC should be considered in patients with a cholestatic pattern of liver test abnormalities, especially in those with underlying IBD. Abnormalities that may be detected on physical examination include jaundice, hepatomegaly, splenomegaly, and excoriations from scratching. […] A diagnosis of PSC is typically established by the demonstration of characteristic multifocal stricturing and dilation of intrahepatic and/or extrahepatic bile ducts on cholangiography. […] The diagnosis of PSC is occasionally made on liver biopsy, which may reveal characteristic features of âonion skin fibrosisâ and fibro-obliterative cholangitis when cholangiography is normal.
#12 Primary Sclerosing Cholangitis Workup: Approach Considerations, Laboratory Studies, Imaging Studies
https://emedicine.medscape.com/article/187724-workup
Liver function tests (LFTs) and histology (liver biopsy) are used in the evaluation of patients with suspected primary sclerosing cholangitis (PSC). […] LFTs, including levels of serum alkaline phosphatase (ALP), serum aminotransferase (ALT and AST), and gamma glutamyl transpeptidase (GGTP), and the presence of hypergammaglobulinemia, are the most valuable in the laboratory workup. […] The most important imaging study is magnetic resonance cholangiopancreatography (MRCP). […] Liver biopsy is rarely used for diagnosis of PSC, although it does help determine both the stage and the prognosis of the disease. […] The most valuable laboratory studies in primary sclerosing cholangitis (PSC) are liver function tests. […] Hypergammaglobulinemia is present in 30% of patients, and 50% have increased immunoglobulin (IgM) levels.
#13 Primary Sclerosing Cholangitis (PSC) – Hepatic and Biliary Disorders – Merck Manual Professional Edition
https://www.merckmanuals.com/professional/hepatic-and-biliary-disorders/gallbladder-and-bile-duct-disorders/primary-sclerosing-cholangitis-psc
Primary sclerosing cholangitis (PSC) is suspected in patients with unexplained abnormalities in liver tests, particularly in those with inflammatory bowel disease. A cholestatic pattern is typical: elevated alkaline phosphatase and gamma-glutamyltransferase (GGT) rather than aminotransferases. […] Diagnosis requires cholangiography to show multiple strictures and dilations in the intrahepatic and extrahepatic bile ducts. Cholangiography should begin with magnetic resonance cholangiopancreatography (MRCP). Endoscopic retrograde cholangiopancreatography (ERCP) is usually a 2nd choice because it is invasive. […] Adults with PSC, even in the absence of cirrhosis, should undergo abdominal imaging (ultrasound, abdominal computed tomography, or magnetic resonance imaging/magnetic resonance cholangiopancreatography) every 6 to 12 months to screen for gallbladder cancer and cholangiocarcinoma. […] Colonoscopy with biopsies should be done in patients without pre-existing inflammatory bowel disease (IBD) at the time of diagnosis of PSC and should be carried out annually in patients with PSC and IBD from the time of diagnosis of PSC due to the increased risk of colorectal adenocarcinoma.
#14 Primary Sclerosing Cholangitis – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK537181/
Primary sclerosing cholangitis (PSC) is a chronic and progressive cholestatic liver disorder of unknown etiology. […] This activity reviews the etiology, pathogenesis, evaluation, and management of primary sclerosing cholangitis and explains the role of the interprofessional team in evaluating and treating patients with this condition. […] Assess how to evaluate for primary sclerosing cholangitis. […] The diagnosis of PSC is typically made with the demonstration of characteristic multiple and focal areas of stricturing and dilation of intrahepatic or extrahepatic bile ducts on cholangiography. […] Diagnostic criteria for the diagnosis of PSC include: An increased serum alkaline phosphatase level that persists for more than 6 months, Cholangiographic findings of bile-duct strictures detected using either MRCP or ERCP, Exclusion of causes of secondary sclerosing cholangitis. […] The key feature in histology is periductal or onion skin fibrosis. […] There is no established treatment for PSC yet. […] The treatment of PSC requires an interprofessional approach.
#15 Primary sclerosing cholangitis – Wikipedia
https://en.wikipedia.org/wiki/Primary_sclerosing_cholangitis
Primary sclerosing cholangitis (PSC) is generally diagnosed on the basis of having at least two of three clinical criteria after secondary causes of sclerosing cholangitis have been ruled out: serum alkaline phosphatase (ALP) 1.5x the upper limit of normal for longer than 6 months […] cholangiography demonstrating biliary strictures or irregularity consistent with PSC […] liver biopsy consistent with PSC (if available). […] Historically, a cholangiogram would be obtained via endoscopic retrograde cholangiopancreatography (ERCP), which typically reveals „beading” (alternating strictures and dilation) of the bile ducts inside and/or outside the liver. Currently, the preferred option for diagnostic cholangiography, given its noninvasive yet highly accurate nature, is magnetic resonance cholangiopancreatography (MRCP), a magnetic resonance imaging technique.
#16 Primary Sclerosing Cholangitis Workup: Approach Considerations, Laboratory Studies, Imaging Studies
https://emedicine.medscape.com/article/187724-workup
Liver function tests (LFTs) and histology (liver biopsy) are used in the evaluation of patients with suspected primary sclerosing cholangitis (PSC). […] LFTs, including levels of serum alkaline phosphatase (ALP), serum aminotransferase (ALT and AST), and gamma glutamyl transpeptidase (GGTP), and the presence of hypergammaglobulinemia, are the most valuable in the laboratory workup. […] The most important imaging study is magnetic resonance cholangiopancreatography (MRCP). […] Liver biopsy is rarely used for diagnosis of PSC, although it does help determine both the stage and the prognosis of the disease. […] The most valuable laboratory studies in primary sclerosing cholangitis (PSC) are liver function tests. […] Hypergammaglobulinemia is present in 30% of patients, and 50% have increased immunoglobulin (IgM) levels.
#17 What is Primary Sclerosing Cholangitis? Diagnosis and Treatment | Prof. Dr. Ãetin Karaca
https://cetinkaraca.com/en/what-is-primary-sclerosing-cholangitis-diagnosis-and-treatment/
There is no characteristic autoantibody test that can be used in PSK. Blood tests show significant increases in serum alkaline phosphatase (AF) and gamma glutamyl trasferase (GGT) levels, while slight increases in aminotransferase levels (ALT and AST). In cases with jaundice, serum bilirubin levels are found to be high. Hypergammaglobulinemia (especially IgM) is seen in 1/3 of the cases. Antinuclear cytoplasmic antibody (ANCA) is positive in blood in 65-85% of cases. […] The diagnosis is made by observing typical findings in a cholangiography to be performed in the presence of clinical and biochemical findings and by distinguishing other diseases that may produce similar findings. Cholangiography (radiological imaging of the biliary tract) is performed with the ERCP procedure and is the most valuable diagnostic method used in the diagnosis of PSC. In cases where ERCP cannot be performed or is undesirable, MRCP is helpful in the diagnosis.
#18 What is Primary Sclerosing Cholangitis? Diagnosis and Treatment | Prof. Dr. Ãetin Karaca
https://cetinkaraca.com/en/what-is-primary-sclerosing-cholangitis-diagnosis-and-treatment/
There is no characteristic autoantibody test that can be used in PSK. Blood tests show significant increases in serum alkaline phosphatase (AF) and gamma glutamyl trasferase (GGT) levels, while slight increases in aminotransferase levels (ALT and AST). In cases with jaundice, serum bilirubin levels are found to be high. Hypergammaglobulinemia (especially IgM) is seen in 1/3 of the cases. Antinuclear cytoplasmic antibody (ANCA) is positive in blood in 65-85% of cases. […] The diagnosis is made by observing typical findings in a cholangiography to be performed in the presence of clinical and biochemical findings and by distinguishing other diseases that may produce similar findings. Cholangiography (radiological imaging of the biliary tract) is performed with the ERCP procedure and is the most valuable diagnostic method used in the diagnosis of PSC. In cases where ERCP cannot be performed or is undesirable, MRCP is helpful in the diagnosis.
#19 Primary Sclerosing Cholangitis â PSC | Choose the Right Test
https://arupconsult.com/content/primary-sclerosing-cholangitis-psc
Liver biopsies are rarely recommended for the diagnosis of primary sclerosing cholangitis (PSC). […] A liver biopsy is necessary to diagnose small-duct PSC and should be performed in patients with normal imaging results who have unexplained cholestatic liver tests. […] A liver biopsy should also be performed if a PSC-autoimmune hepatitis (AIH) variant form or immunoglobulin G4 (IgG4)-related sclerosing cholangitis is suspected. […] Although there is no specific biochemical profile for PSC, liver biochemistry is abnormal in 75% of patients with PSC, and a biochemical profile may be useful in the diagnostic process. […] In certain studies, autoantibodies have been found in more than 50% of patients with PSC; however, due to a lack of specific autoantibodies associated with PSC, they play only a minor role in diagnosis.
#20 Primary sclerosing cholangitis – Wikipedia
https://en.wikipedia.org/wiki/Primary_sclerosing_cholangitis
Most people with PSC have evidence of autoantibodies and abnormal immunoglobulin levels. For example, approximately 80% of people with PSC have perinuclear antineutrophil cytoplasmic antibodies (P-ANCA); however, this and other immunoglobulin findings are not specific to those with PSC and are of unclear clinical significance/consequence. […] The differential diagnosis can include primary biliary cholangitis (formerly referred to as primary biliary cirrhosis), drug-induced cholestasis, cholangiocarcinoma, IgG4-related disease, post-liver transplantation nonanastomotic biliary strictures, and HIV-associated cholangiopathy. […] Primary sclerosing cholangitis and primary biliary cholangitis are distinct entities and exhibit important differences, including the site of tissue damage within the liver, associations with IBD, which includes ulcerative colitis and Crohn’s disease, response to treatment, and risks of disease progression.
#21 Primary sclerosing cholangitis | Radiology Reference Article | Radiopaedia.org
https://radiopaedia.org/articles/primary-sclerosing-cholangitis?lang=us
Primary sclerosing cholangitis (PSC) is an uncommon inflammatory condition that affects the biliary tree resulting in multiple strictures, liver damage, and eventually cirrhosis. […] The diagnosis can be made when there are classical imaging features in the correct clinical context, and secondary causes of cholangitis have been excluded. […] Most patients demonstrate at least one type of autoantibody, typically anti-smooth muscle antibody (ASMA) and antinuclear antibody (ANA), which are seen in up to 75%. p-ANCA is also positive in up to 80% of patients with primary sclerosing cholangitis. […] ERCP has traditionally been the gold standard for the depiction of the biliary tree, and also offers the ability to perform cholangioplasty, if necessary. In practice, however, MRCP is used before ERCP since it is non-invasive, can assess the liver, and avoids the 10% risk of hospitalization from ERCP in primary sclerosing cholangitis patients. […] The characteristic findings on direct imaging of the biliary tree are multiple segmental strictures, typically short segment, intervening segments are of normal caliber or slightly dilated (beading).
#22 Primary sclerosing cholangitis – Rabiee – Translational Gastroenterology and Hepatology
https://tgh.amegroups.org/article/view/6491/html
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease that is characterized by intra- and/or extrahepatic bile duct injury. The clinical presentation of PSC correlates with the sequence of inflammatory bile duct destruction and fibrosis, which results in bile duct stricturing, cholestasis, and eventually biliary cirrhosis with end-stage liver disease and hepatic dysfunction. PSC is increasingly diagnosed early in the stage of the disease course, and, as a result, the majority of patients do not have any clinical symptoms at the time of diagnosis. In the majority of cases, the diagnosis of PSC is prompted by the finding of cholestasis at the time of routine health evaluation or screening of high-risk patients such as those with inflammatory bowel disease (IBD). […] Currently, no autoimmune antibodies are sufficiently specific in the diagnosis of PSC. The most commonly positive autoantibodies are the perinuclear antineutrophilic autoantibodies (pANCA), which are found in approximately 80% of patients but lack diagnostic specificity. The diagnostic modality of choice for the diagnosis of PSC is magnetic resonance cholangiopancreatography (MRCP), which has been shown in a meta-analysis to have a sensitivity of 86% and specificity of 94% when compared to endoscopic retrograde cholangiopancreatography (ERCP). The diagnosis of PSC is typically established in patients with chronic cholestasis when cholangiographic studies (MRCP, ERCP, or percutaneous transhepatic cholangiogram) show characteristic features of PSC, and secondary forms of sclerosing cholangitis are excluded.
#23 Primary sclerosing cholangitis | Radiology Reference Article | Radiopaedia.org
https://radiopaedia.org/articles/primary-sclerosing-cholangitis?lang=us
Primary sclerosing cholangitis (PSC) is an uncommon inflammatory condition that affects the biliary tree resulting in multiple strictures, liver damage, and eventually cirrhosis. […] The diagnosis can be made when there are classical imaging features in the correct clinical context, and secondary causes of cholangitis have been excluded. […] Most patients demonstrate at least one type of autoantibody, typically anti-smooth muscle antibody (ASMA) and antinuclear antibody (ANA), which are seen in up to 75%. p-ANCA is also positive in up to 80% of patients with primary sclerosing cholangitis. […] ERCP has traditionally been the gold standard for the depiction of the biliary tree, and also offers the ability to perform cholangioplasty, if necessary. In practice, however, MRCP is used before ERCP since it is non-invasive, can assess the liver, and avoids the 10% risk of hospitalization from ERCP in primary sclerosing cholangitis patients. […] The characteristic findings on direct imaging of the biliary tree are multiple segmental strictures, typically short segment, intervening segments are of normal caliber or slightly dilated (beading).
#24 Primary Sclerosing Cholangitis Workup: Approach Considerations, Laboratory Studies, Imaging Studies
https://emedicine.medscape.com/article/187724-workup
Liver function tests (LFTs) and histology (liver biopsy) are used in the evaluation of patients with suspected primary sclerosing cholangitis (PSC). […] LFTs, including levels of serum alkaline phosphatase (ALP), serum aminotransferase (ALT and AST), and gamma glutamyl transpeptidase (GGTP), and the presence of hypergammaglobulinemia, are the most valuable in the laboratory workup. […] The most important imaging study is magnetic resonance cholangiopancreatography (MRCP). […] Liver biopsy is rarely used for diagnosis of PSC, although it does help determine both the stage and the prognosis of the disease. […] The most valuable laboratory studies in primary sclerosing cholangitis (PSC) are liver function tests. […] Hypergammaglobulinemia is present in 30% of patients, and 50% have increased immunoglobulin (IgM) levels.
#25 Primary Sclerosing Cholangitis â PSC | Choose the Right Test
https://arupconsult.com/content/primary-sclerosing-cholangitis-psc
Because antimitochondrial antibodies (AMAs) are a hallmark of PBC but are absent in PSC, AMA testing may be useful to differentiate between the two ALDs. […] Although there are no established prognostic tools for PSC, a patients biochemical profile at the time of diagnosis may inform prognosis. […] An increased serum immunoglobulin G4 (IgG4) may correlate with a more severe disease course. […] The most commonly used model is the revised Mayo natural history model for PSC, which uses age, bilirubin level, albumin level, AST level, and history of variceal bleeding to predict a patients probability of survival. […] Regular screening should be performed for other disorders commonly associated with PSC. […] Because of the high prevalence of IBD in patients with PSC, patients should undergo a full colonoscopy with biopsies at the time of diagnosis to screen for associated IBD.
#26 Primary Sclerosing Cholangitis – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK537181/
Primary sclerosing cholangitis (PSC) is a chronic and progressive cholestatic liver disorder of unknown etiology. […] This activity reviews the etiology, pathogenesis, evaluation, and management of primary sclerosing cholangitis and explains the role of the interprofessional team in evaluating and treating patients with this condition. […] Assess how to evaluate for primary sclerosing cholangitis. […] The diagnosis of PSC is typically made with the demonstration of characteristic multiple and focal areas of stricturing and dilation of intrahepatic or extrahepatic bile ducts on cholangiography. […] Diagnostic criteria for the diagnosis of PSC include: An increased serum alkaline phosphatase level that persists for more than 6 months, Cholangiographic findings of bile-duct strictures detected using either MRCP or ERCP, Exclusion of causes of secondary sclerosing cholangitis. […] The key feature in histology is periductal or onion skin fibrosis. […] There is no established treatment for PSC yet. […] The treatment of PSC requires an interprofessional approach.
#27
https://link.springer.com/article/10.1007/s11894-006-0067-8
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease caused by progressive inflammatory destruction of intrahepatic and extrahepatic bile ducts, and ultimately cirrhosis. […] Cholangiography is the gold standard for diagnosis of PSC. The typical radiologic findings include multifocal strictures and dilation involving the intrahepatic or extrahepatic biliary tract, or both. […] Liver transplantation is a good option for patients with advanced PSC, although the disease can recur after successful transplantation.
#28 Diagnosis of Primary Sclerosing Cholangitis – NIDDK
https://www.niddk.nih.gov/health-information/liver-disease/primary-sclerosing-cholangitis/diagnosis
Doctors diagnose primary sclerosing cholangitis (PSC) based on medical and family history, a physical exam, and the results of medical tests. […] The doctor will ask about your symptoms. […] Your doctor will examine your body, including your abdomen. […] Liver tests can show abnormal levels of liver enzymes and other substances in your blood. Abnormal levels of certain liver enzymes may be a sign your liver or bile ducts are damaged. […] To diagnose PSC, doctors typically order a special imaging test to examine the bile ducts, such as magnetic resonance cholangiopancreatography (MRCP), which uses a magnetic resonance imaging (MRI) machine to create pictures of the bile ducts. MRCP is the most common test that doctors use to diagnose PSC. […] A liver biopsy is generally not needed to diagnose PSC. However, in some cases, doctors may order a liver biopsy to check for signs of other liver diseases, such as autoimmune hepatitis. […] For people who have PSC and haven’t already been diagnosed with IBD, doctors may recommend a colonoscopy to check for IBD.
#29 Primary Sclerosing Cholangitis: Symptoms, Treatment & Diagnosis
https://my.clevelandclinic.org/health/diseases/23569-primary-sclerosing-cholangitis
Magnetic resonance cholangiopancreatography (MRCP). This test uses magnetic resonance imaging (MRI) to produce detailed pictures of the biliary tree (your liver, gallbladder and bile ducts). This is the first-line imaging test for PSC because its non-invasive and avoids radiation exposure. Occasionally, it might not reveal an early or mild case of the disease, and you might need another kind of imaging test.
#30 Primary sclerosing cholangitis – Rabiee – Translational Gastroenterology and Hepatology
https://tgh.amegroups.org/article/view/6491/html
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease that is characterized by intra- and/or extrahepatic bile duct injury. The clinical presentation of PSC correlates with the sequence of inflammatory bile duct destruction and fibrosis, which results in bile duct stricturing, cholestasis, and eventually biliary cirrhosis with end-stage liver disease and hepatic dysfunction. PSC is increasingly diagnosed early in the stage of the disease course, and, as a result, the majority of patients do not have any clinical symptoms at the time of diagnosis. In the majority of cases, the diagnosis of PSC is prompted by the finding of cholestasis at the time of routine health evaluation or screening of high-risk patients such as those with inflammatory bowel disease (IBD). […] Currently, no autoimmune antibodies are sufficiently specific in the diagnosis of PSC. The most commonly positive autoantibodies are the perinuclear antineutrophilic autoantibodies (pANCA), which are found in approximately 80% of patients but lack diagnostic specificity. The diagnostic modality of choice for the diagnosis of PSC is magnetic resonance cholangiopancreatography (MRCP), which has been shown in a meta-analysis to have a sensitivity of 86% and specificity of 94% when compared to endoscopic retrograde cholangiopancreatography (ERCP). The diagnosis of PSC is typically established in patients with chronic cholestasis when cholangiographic studies (MRCP, ERCP, or percutaneous transhepatic cholangiogram) show characteristic features of PSC, and secondary forms of sclerosing cholangitis are excluded.
#31 IBD and Primary Sclerosing Cholangitis (PSC)
https://www.verywellhealth.com/ibd-and-primary-sclerosing-cholangitis-psc-1941767
A liver biopsy can be useful when the imaging modalities are not diagnostic or when overlap syndrome is suspected. […] Lastly, an MRCP, specialized magnetic resonance imaging, can be used to detect the rare condition. A 2019 medical study explains that the noninvasive imaging does not require X-rays like an ERCP, so there is no radiation exposure to the patient. „MRCP has high diagnostic sensitivity (86%) and specificity (94%) for detection of PSC,” according to the study.
#32 British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis | Gut
https://gut.bmj.com/content/68/8/1356
These guidelines on the management of primary sclerosing cholangitis (PSC) were commissioned by the British Society of Gastroenterology liver section. […] The guidelines aim to support general physicians, gastroenterologists and surgeons in managing adults with PSC or those presenting with similar cholangiopathies which may mimic PSC, such as IgG4 sclerosing cholangitis. […] Diagnosis is based on the cholangiographic (or histological) features of sclerosing cholangitis in the absence of identifiable causes of secondary sclerosing cholangitis. […] Non-invasive investigations such as magnetic resonance cholangiopancreatography (MRCP), dynamic liver MRI and/or contrast CT should be performed in patients who have new or changing symptoms or evolving abnormalities in laboratory investigations.
#33 Primary Sclerosing Cholangitis (PSC) – Guts UK
https://gutscharity.org.uk/advice-and-information/conditions/primary-sclerosing-cholangitis-psc/
Primary Sclerosing Cholangitis (PSC) is a rare, immune related disease that affects bile ducts and liver. […] There is no single diagnostic blood test for PSC. This means that your doctors need to piece together information from your medical history, blood tests, symptoms, colonoscopy and scans to make a diagnosis. […] The gold standard tool for diagnosing Primary Sclerosing Cholangitis is an MRCP (magnetic resonance cholangiopancreatography). An MRCP is a type of MRI scan that looks closely at the bile ducts. It does not involve ionizing radiation. […] Liver biopsies are not generally needed. But if the MRCP is normal and PSC is still a possibility, then a liver biopsy can be helpful in making the diagnosis. […] In many people without symptoms, a PSC diagnosis is made by chance, after routine tests for IBD show abnormal liver blood results. […] Because PSC is rare and there are no currently available treatments, everybody who is diagnosed or suspected to have PSC should be offered discussion with a PSC specialist. Plus, access to clinical trials of new medicines, regular monitoring (as outlined above) and surveillance.
#34 Primary sclerosing cholangitis | Radiology Reference Article | Radiopaedia.org
https://radiopaedia.org/articles/primary-sclerosing-cholangitis?lang=us
Primary sclerosing cholangitis (PSC) is an uncommon inflammatory condition that affects the biliary tree resulting in multiple strictures, liver damage, and eventually cirrhosis. […] The diagnosis can be made when there are classical imaging features in the correct clinical context, and secondary causes of cholangitis have been excluded. […] Most patients demonstrate at least one type of autoantibody, typically anti-smooth muscle antibody (ASMA) and antinuclear antibody (ANA), which are seen in up to 75%. p-ANCA is also positive in up to 80% of patients with primary sclerosing cholangitis. […] ERCP has traditionally been the gold standard for the depiction of the biliary tree, and also offers the ability to perform cholangioplasty, if necessary. In practice, however, MRCP is used before ERCP since it is non-invasive, can assess the liver, and avoids the 10% risk of hospitalization from ERCP in primary sclerosing cholangitis patients. […] The characteristic findings on direct imaging of the biliary tree are multiple segmental strictures, typically short segment, intervening segments are of normal caliber or slightly dilated (beading).
#35 Primary Sclerosing Cholangitis Workup: Approach Considerations, Laboratory Studies, Imaging Studies
https://emedicine.medscape.com/article/187724-workup
Because it is noninvasive, magnetic resonance cholangiopancreatography (MRCP) is the preferred study to evaluate the bile ducts. […] Although endoscopic retrograde cholangiopancreatography (ERCP) is the gold standard for diagnosis of cholangitis, it is used more for therapeutic intervention than for confirming the diagnosis of primary sclerosing cholangitis (PSC). […] Liver biopsy is not required for the diagnosis of primary sclerosing cholangitis (PSC) if magnetic resonance cholangiopancreatography (MRCP) findings are suggestive.
#36 British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis | Gut
https://gut.bmj.com/content/68/8/1356
We recommend that MRCP should be the principal imaging modality for the investigation of suspected PSC. […] We recommend that liver biopsy is normally reserved for possible small duct PSC, assessment of suspected possible overlap variants or instances where the diagnosis is unclear. […] We recommend that patients with PSC should ordinarily not undergo ERCP until there has been expert multidisciplinary assessment to justify endoscopic intervention. […] We recommend that colitis should be sought in all patients with PSC using colonoscopy and colonic biopsies. […] We recommend that patients with suspected PSC undergoing ERCP should receive prophylactic antibiotics. […] We recommend that non-invasive investigations such as MRCP, dynamic liver MRI and/or contrast CT should be performed in patients who have new or changing symptoms or evolving abnormalities in laboratory investigations.
#37 British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis | Gut
https://gut.bmj.com/content/68/8/1356
We recommend that patients with PSC should ordinarily not undergo ERCP until there has been expert multidisciplinary assessment to justify endoscopic intervention. […] We recommend that in patients undergoing ERCP for dominant strictures, pathological sampling of suspicious strictures is mandatory. […] We recommend that in patients undergoing ERCP for dominant strictures, biliary dilatation is preferred to the insertion of biliary stents. […] We recommend that patients with symptomatic, evolving or complex disease should be referred for expert multidisciplinary assessment. […] We recommend that all patients with PSC should have a risk assessment for osteoporosis. […] We recommend that in patients with fatigue, alternative causes should be actively sought and treated. […] We recommend that when a diagnosis of cholangiocarcinoma is clinically suspected, referral for specialist multidisciplinary meeting (MDM) review is essential.
#38 Diagnosis and management of primary sclerosing cholangitis-perspectives from a therapeutic endoscopist
https://www.wjgnet.com/1948-5182/full/v7/i5/799.htm
The endoscopist is also consulted when these patients develop worsening liver function tests on follow-up. Screening these patients for the development of dominant strictures and treating them constitutes the responsibility of a therapeutic endoscopist. […] A therapeutic endoscopist plays an integral part in the diagnosis and management of PSC patients and the endoscopist forms an important pillar in the therapeutic armamentarium of patients with PSC. […] Brush cytology obtained during ERCP and use of fluorescence in situ hybridization forms the initial diagnostic step in the investigation of patients with dominant biliary strictures. […] The presence of worsening symptoms in patients with PSC typically warrants investigation to exclude a dominant extrahepatic biliary stricture. […] A dominant stricture has been defined as a stenosis with a diameter of 1.5 mm in the common bile duct or of 1 mm in the hepatic duct.
#39 Primary Sclerosing Cholangitis (Causes, Symptoms and Treatment)
https://patient.info/doctor/primary-sclerosing-cholangitis-pro
Strictures causing recurrent cholangitis can be treated by balloon dilatation (endoscopic or percutaneous). […] Liver transplantation is an effective treatment. The indications for liver transplantation are similar to other causes of chronic liver disease but also include intractable pruritus and recurrent cholangitis. […] PSC is a progressive disease of the liver characterised by inflammation and destruction of the intrahepatic and/or extrahepatic bile ducts, leading to fibrosis and ultimately liver failure, cirrhosis and an increased risk of malignancy. […] Prognosis after liver transplant: Survival post-transplant is about 94% 1 year, 86% at 5 years and 70% at 10 years.
#40 The best approach for diagnosing primary sclerosing cholangitis | Clinics
https://www.elsevier.es/en-revista-clinics-22-articulo-the-best-approach-for-diagnosing-S180759322202316X
In recent years, cholangio-NMR has undergone a significant technical evolution and is a preferred tool to investigate cholestatic disease. […] However, ERCP remains a valuable therapeutic method to manipulate the biliary tree (removal of sludge and stones and the dilation of the duct). […] ERCP with papillotomy may complicate cholestatic liver disease. Because other non-invasive tests such as cholangio-NMR are currently widely available, we believe that ERCP has a limited role in the diagnosis of PSC and should be indicated only for therapeutic purposes in patients with PSC.
#41 Primary sclerosing cholangitis – Knowledge @ AMBOSS
https://www.amboss.com/us/knowledge/primary-sclerosing-cholangitis/
Magnetic resonance cholangiopancreatography is the method of choice (if there is no biliary obstruction). […] Endoscopic retrograde cholangiopancreatography should not be used for diagnosis of PSC except in the following scenarios: Need for simultaneous therapeutic intervention for biliary obstruction, Contraindications to MRI. […] Not routinely indicated as part of the workup for PSC. […] Consider in select patients to evaluate for: Small-duct PSC, Autoimmune hepatitis-PSC overlap syndrome.
#42 Primary Sclerosing Cholangitis (Causes, Symptoms and Treatment)
https://patient.info/doctor/primary-sclerosing-cholangitis-pro
Primary sclerosing cholangitis (PSC) is a chronic cholestatic disorder characterised by inflammation and fibrosis of intrahepatic and extrahepatic bile ducts, resulting in multifocal biliary strictures. […] Ultrasound is the initial investigation and may show bile duct dilatation and liver and splenic changes; however, it is not diagnostic for PSC. […] Magnetic resonance cholangiopancreatography (MRCP) is now the standard procedure to visualise the intrahepatic and extrahepatic bile ducts. […] Liver biopsy is rarely diagnostic but may be useful for staging PSC. […] Despite years of research, medical treatments are only at best able to help manage primary sclerosing cholangitis symptoms. […] Liver transplantation remains the only life-prolonging treatment for patients with end-stage disease.
#43 Primary Sclerosing Cholangitis
https://austinpublishinggroup.com/radiology/fulltext/ajr-v3-id1057.php
We present a case of a 47-year-old woman with non-specific symptoms and signs of anaemia. […] Diagnosis is achieved by imaging, laboratory markers and histology. […] The diagnosis can be made on imaging by identifying the typical cholangiographic findings coupled with clinical presentation. […] The role of ultrasound is very limited and can detect changes of cirrhosis only in advanced stage. […] ERCP is the gold standard examination and can be used for diagnosis as well as treatment of strictures and/or removal of debris within the ducts. […] Magnetic Resonance Cholangio-Pancreaticography (MRCP) is becoming investigation of choice due to inherent advantages over ERCP. […] The advantages of MRCP apart from being non-invasive modality, include lack of radiation, faster imaging and visualization of dilated peripheral intrahepatic ducts.
#44 British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis | Gut
https://gut.bmj.com/content/68/8/1356
We recommend that where cholangiocarcinoma is suspected, contrast-enhanced, cross-sectional imaging remains the initial preferred investigation for diagnosis and staging. […] We recommend that patients with PSC who have coexistent colonic inflammatory bowel disease (IBD) should have annual colonoscopic surveillance from the time of diagnosis of colitis in line with the British Society of Gastroenterology (BSG) guidelines. […] We recommend that eligibility and referral should be assessed in line with the national guidelines.
#45 Diagnosis of Primary Sclerosing Cholangitis – NIDDK
https://www.niddk.nih.gov/health-information/liver-disease/primary-sclerosing-cholangitis/diagnosis
Doctors diagnose primary sclerosing cholangitis (PSC) based on medical and family history, a physical exam, and the results of medical tests. […] The doctor will ask about your symptoms. […] Your doctor will examine your body, including your abdomen. […] Liver tests can show abnormal levels of liver enzymes and other substances in your blood. Abnormal levels of certain liver enzymes may be a sign your liver or bile ducts are damaged. […] To diagnose PSC, doctors typically order a special imaging test to examine the bile ducts, such as magnetic resonance cholangiopancreatography (MRCP), which uses a magnetic resonance imaging (MRI) machine to create pictures of the bile ducts. MRCP is the most common test that doctors use to diagnose PSC. […] A liver biopsy is generally not needed to diagnose PSC. However, in some cases, doctors may order a liver biopsy to check for signs of other liver diseases, such as autoimmune hepatitis. […] For people who have PSC and haven’t already been diagnosed with IBD, doctors may recommend a colonoscopy to check for IBD.
#46 Primary Sclerosing Cholangitis Workup: Approach Considerations, Laboratory Studies, Imaging Studies
https://emedicine.medscape.com/article/187724-workup
Because it is noninvasive, magnetic resonance cholangiopancreatography (MRCP) is the preferred study to evaluate the bile ducts. […] Although endoscopic retrograde cholangiopancreatography (ERCP) is the gold standard for diagnosis of cholangitis, it is used more for therapeutic intervention than for confirming the diagnosis of primary sclerosing cholangitis (PSC). […] Liver biopsy is not required for the diagnosis of primary sclerosing cholangitis (PSC) if magnetic resonance cholangiopancreatography (MRCP) findings are suggestive.
#47 Primary Sclerosing Cholangitis â PSC | Choose the Right Test
https://arupconsult.com/content/primary-sclerosing-cholangitis-psc
Liver biopsies are rarely recommended for the diagnosis of primary sclerosing cholangitis (PSC). […] A liver biopsy is necessary to diagnose small-duct PSC and should be performed in patients with normal imaging results who have unexplained cholestatic liver tests. […] A liver biopsy should also be performed if a PSC-autoimmune hepatitis (AIH) variant form or immunoglobulin G4 (IgG4)-related sclerosing cholangitis is suspected. […] Although there is no specific biochemical profile for PSC, liver biochemistry is abnormal in 75% of patients with PSC, and a biochemical profile may be useful in the diagnostic process. […] In certain studies, autoantibodies have been found in more than 50% of patients with PSC; however, due to a lack of specific autoantibodies associated with PSC, they play only a minor role in diagnosis.
#48 Primary Sclerosing Cholangitis: Diagnostic Criteria
https://www.mdpi.com/2379-139X/10/1/5
The diagnosis of large duct PSC is mostly based on the presence of elevation of cholestatic indices and the association of typical cholangiographic findings, i.e., the presence of strictures and dilatations in the intra- and/or extrahepatic bile ducts after the exclusion of causes of secondary sclerosing cholangitis. […] Liver biopsy is not routinely performed for the diagnosis of large duct PSC but is mandatory in the case of suspicion of small duct PSC or overlap with autoimmune hepatitis.
#49 British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis | Gut
https://gut.bmj.com/content/68/8/1356
We recommend that MRCP should be the principal imaging modality for the investigation of suspected PSC. […] We recommend that liver biopsy is normally reserved for possible small duct PSC, assessment of suspected possible overlap variants or instances where the diagnosis is unclear. […] We recommend that patients with PSC should ordinarily not undergo ERCP until there has been expert multidisciplinary assessment to justify endoscopic intervention. […] We recommend that colitis should be sought in all patients with PSC using colonoscopy and colonic biopsies. […] We recommend that patients with suspected PSC undergoing ERCP should receive prophylactic antibiotics. […] We recommend that non-invasive investigations such as MRCP, dynamic liver MRI and/or contrast CT should be performed in patients who have new or changing symptoms or evolving abnormalities in laboratory investigations.
#50 Primary sclerosing cholangitis – Knowledge @ AMBOSS
https://www.amboss.com/us/knowledge/primary-sclerosing-cholangitis/
Magnetic resonance cholangiopancreatography is the method of choice (if there is no biliary obstruction). […] Endoscopic retrograde cholangiopancreatography should not be used for diagnosis of PSC except in the following scenarios: Need for simultaneous therapeutic intervention for biliary obstruction, Contraindications to MRI. […] Not routinely indicated as part of the workup for PSC. […] Consider in select patients to evaluate for: Small-duct PSC, Autoimmune hepatitis-PSC overlap syndrome.
#51 Pathology Pearls Post 8: Primary Sclerosing Cholangitis (PSC) | AASLD
https://www.aasld.org/liver-fellow-network/core-series/pathology-pearls/pathology-pearls-post-8-primary-sclerosing
Given the absence of etiology for secondary cholangitis such as infection and neoplasia, the histologic findings are suggestive of primary sclerosing cholangitis (PSC) […] Although the gold standard for the diagnosis of PSC remains endoscopic retrograde cholangiopancreatography (ERCP) / magnetic resonance cholangiopancreatography (MRCP), a liver biopsy can aid in the diagnosis. […] The most classic findings of PSC include large bile ducts surrounded by concentric fibrosis, which is also referred to as onion skin fibrosis. […] When affected large bile ducts are not sampled, findings in liver biopsies from patients with PSC may be nonspecific and can demonstrate portal tracts with increased inflammation and fibrosis. […] Primary biliary cholangitis (PBC) should also be considered in the differential diagnosis of PSC. […] Although less common, IgG4-associated cholangitis can mimic PSC.
#52 Primary Sclerosing Cholangitis (PSC)
https://www.uchealth.com/en/conditions/primary-sclerosing-cholangitis
Diagnosing PSC requires a thorough approach, including a review of symptoms, medical history, and specialized testing to confirm the presence and extent of bile duct damage. […] Blood Tests: Liver function tests check for elevated liver enzymes, and autoimmune markers may help identify PSC as part of autoimmune disease. […] Magnetic Resonance Cholangiopancreatography (MRCP): This imaging technique provides detailed images of the bile ducts, helping to detect bile duct narrowing, blockages, and liver damage. […] Endoscopic Retrograde Cholangiopancreatography (ERCP): Used for diagnostic and therapeutic purposes, ERCP can help identify and treat bile duct strictures. […] Liver Biopsy: Sometimes performed to assess liver tissue for fibrosis and rule out other liver diseases, such as primary biliary cholangitis or autoimmune hepatitis. […] Regular follow-ups and imaging allow healthcare providers to track disease progression and manage complications.
#53 Primary sclerosing cholangitis – Rabiee – Translational Gastroenterology and Hepatology
https://tgh.amegroups.org/article/view/6491/html
The histological hallmark of PSC is the finding of concentric periductal fibrosis, also known as onion-skin fibrosis, but it is only detected in less than 15% of liver biopsies of patients with PSC. Liver biopsy is not necessary to diagnose PSC, unless there is suspicion for small duct PSC or PSC with features of AIH.
#54 Pathology Pearls Post 8: Primary Sclerosing Cholangitis (PSC) | AASLD
https://www.aasld.org/liver-fellow-network/core-series/pathology-pearls/pathology-pearls-post-8-primary-sclerosing
Given the absence of etiology for secondary cholangitis such as infection and neoplasia, the histologic findings are suggestive of primary sclerosing cholangitis (PSC) […] Although the gold standard for the diagnosis of PSC remains endoscopic retrograde cholangiopancreatography (ERCP) / magnetic resonance cholangiopancreatography (MRCP), a liver biopsy can aid in the diagnosis. […] The most classic findings of PSC include large bile ducts surrounded by concentric fibrosis, which is also referred to as onion skin fibrosis. […] When affected large bile ducts are not sampled, findings in liver biopsies from patients with PSC may be nonspecific and can demonstrate portal tracts with increased inflammation and fibrosis. […] Primary biliary cholangitis (PBC) should also be considered in the differential diagnosis of PSC. […] Although less common, IgG4-associated cholangitis can mimic PSC.
#55 Primary Sclerosing Cholangitis – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK537181/
Primary sclerosing cholangitis (PSC) is a chronic and progressive cholestatic liver disorder of unknown etiology. […] This activity reviews the etiology, pathogenesis, evaluation, and management of primary sclerosing cholangitis and explains the role of the interprofessional team in evaluating and treating patients with this condition. […] Assess how to evaluate for primary sclerosing cholangitis. […] The diagnosis of PSC is typically made with the demonstration of characteristic multiple and focal areas of stricturing and dilation of intrahepatic or extrahepatic bile ducts on cholangiography. […] Diagnostic criteria for the diagnosis of PSC include: An increased serum alkaline phosphatase level that persists for more than 6 months, Cholangiographic findings of bile-duct strictures detected using either MRCP or ERCP, Exclusion of causes of secondary sclerosing cholangitis. […] The key feature in histology is periductal or onion skin fibrosis. […] There is no established treatment for PSC yet. […] The treatment of PSC requires an interprofessional approach.
#56 Primary sclerosing cholangitis – Wikipedia
https://en.wikipedia.org/wiki/Primary_sclerosing_cholangitis
Primary sclerosing cholangitis (PSC) is generally diagnosed on the basis of having at least two of three clinical criteria after secondary causes of sclerosing cholangitis have been ruled out: serum alkaline phosphatase (ALP) 1.5x the upper limit of normal for longer than 6 months […] cholangiography demonstrating biliary strictures or irregularity consistent with PSC […] liver biopsy consistent with PSC (if available). […] Historically, a cholangiogram would be obtained via endoscopic retrograde cholangiopancreatography (ERCP), which typically reveals „beading” (alternating strictures and dilation) of the bile ducts inside and/or outside the liver. Currently, the preferred option for diagnostic cholangiography, given its noninvasive yet highly accurate nature, is magnetic resonance cholangiopancreatography (MRCP), a magnetic resonance imaging technique.
#57 Primary Sclerosing Cholangitis – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK537181/
Primary sclerosing cholangitis (PSC) is a chronic and progressive cholestatic liver disorder of unknown etiology. […] This activity reviews the etiology, pathogenesis, evaluation, and management of primary sclerosing cholangitis and explains the role of the interprofessional team in evaluating and treating patients with this condition. […] Assess how to evaluate for primary sclerosing cholangitis. […] The diagnosis of PSC is typically made with the demonstration of characteristic multiple and focal areas of stricturing and dilation of intrahepatic or extrahepatic bile ducts on cholangiography. […] Diagnostic criteria for the diagnosis of PSC include: An increased serum alkaline phosphatase level that persists for more than 6 months, Cholangiographic findings of bile-duct strictures detected using either MRCP or ERCP, Exclusion of causes of secondary sclerosing cholangitis. […] The key feature in histology is periductal or onion skin fibrosis. […] There is no established treatment for PSC yet. […] The treatment of PSC requires an interprofessional approach.
#58 Primary Sclerosing Cholangitis: Diagnostic Criteria
https://www.mdpi.com/2379-139X/10/1/5
The diagnosis of large duct PSC is mostly based on the presence of elevation of cholestatic indices and the association of typical cholangiographic findings, i.e., the presence of strictures and dilatations in the intra- and/or extrahepatic bile ducts after the exclusion of causes of secondary sclerosing cholangitis. […] Liver biopsy is not routinely performed for the diagnosis of large duct PSC but is mandatory in the case of suspicion of small duct PSC or overlap with autoimmune hepatitis.
#59 Primary Sclerosing Cholangitis – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK537181/
Primary sclerosing cholangitis (PSC) is a chronic and progressive cholestatic liver disorder of unknown etiology. […] This activity reviews the etiology, pathogenesis, evaluation, and management of primary sclerosing cholangitis and explains the role of the interprofessional team in evaluating and treating patients with this condition. […] Assess how to evaluate for primary sclerosing cholangitis. […] The diagnosis of PSC is typically made with the demonstration of characteristic multiple and focal areas of stricturing and dilation of intrahepatic or extrahepatic bile ducts on cholangiography. […] Diagnostic criteria for the diagnosis of PSC include: An increased serum alkaline phosphatase level that persists for more than 6 months, Cholangiographic findings of bile-duct strictures detected using either MRCP or ERCP, Exclusion of causes of secondary sclerosing cholangitis. […] The key feature in histology is periductal or onion skin fibrosis. […] There is no established treatment for PSC yet. […] The treatment of PSC requires an interprofessional approach.
#60 Primary sclerosing cholangitis | Orphanet Journal of Rare Diseases | Full Text
https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-1-41
Differential diagnosis should exclude other liver diseases such as autoimmune hepatitis (can coexist with PSC, termed overlap syndrome), primary biliary cirrhosis, viral hepatitis, as well as secondary causes of cholangitis. […] Patients who have cholestatic liver function tests and a clinical diagnosis of PSC with features consistent with this diagnosis on the liver biopsy, but who have a normal cholangiogram, have small duct PSC; 611% of the PSC population have small duct disease.
#61 What is Primary Sclerosing Cholangitis? Diagnosis and Treatment | Prof. Dr. Ãetin Karaca
https://cetinkaraca.com/en/what-is-primary-sclerosing-cholangitis-diagnosis-and-treatment/
In up to 10% of patients without an abnormality in cholangiography, the appearance of onion skin can be detected in the small bile ducts in the liver biopsy. This condition is called small duct Primary sclerosing cholangitis (small-duct PSC). This type of the disease has a better prognosis than the classical type.
#62 Diagnosis of Primary Sclerosing Cholangitis – NIDDK
https://www.niddk.nih.gov/health-information/liver-disease/primary-sclerosing-cholangitis/diagnosis
Doctors diagnose primary sclerosing cholangitis (PSC) based on medical and family history, a physical exam, and the results of medical tests. […] The doctor will ask about your symptoms. […] Your doctor will examine your body, including your abdomen. […] Liver tests can show abnormal levels of liver enzymes and other substances in your blood. Abnormal levels of certain liver enzymes may be a sign your liver or bile ducts are damaged. […] To diagnose PSC, doctors typically order a special imaging test to examine the bile ducts, such as magnetic resonance cholangiopancreatography (MRCP), which uses a magnetic resonance imaging (MRI) machine to create pictures of the bile ducts. MRCP is the most common test that doctors use to diagnose PSC. […] A liver biopsy is generally not needed to diagnose PSC. However, in some cases, doctors may order a liver biopsy to check for signs of other liver diseases, such as autoimmune hepatitis. […] For people who have PSC and haven’t already been diagnosed with IBD, doctors may recommend a colonoscopy to check for IBD.
#63 Primary sclerosing cholangitis – Symptoms and causes – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/primary-sclerosing-cholangitis/symptoms-causes/syc-20355797
Primary sclerosing cholangitis is often diagnosed before symptoms appear when a routine blood test or an X-ray taken for an unrelated condition shows liver abnormalities. […] If primary sclerosing cholangitis is diagnosed, it’s important to look for inflammatory bowel disease because there is a greater risk of colon cancer. […] Primary sclerosing cholangitis and inflammatory bowel disease don’t always appear at the same time, though. In some cases, primary sclerosing cholangitis is present for years before inflammatory bowel disease occurs. […] Your doctor may recommend testing for inflammatory bowel disease, even if you have no signs or symptoms, since the risk of colon cancer is elevated if you have both diseases.
#64 British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis | Gut
https://gut.bmj.com/content/68/8/1356
We recommend that MRCP should be the principal imaging modality for the investigation of suspected PSC. […] We recommend that liver biopsy is normally reserved for possible small duct PSC, assessment of suspected possible overlap variants or instances where the diagnosis is unclear. […] We recommend that patients with PSC should ordinarily not undergo ERCP until there has been expert multidisciplinary assessment to justify endoscopic intervention. […] We recommend that colitis should be sought in all patients with PSC using colonoscopy and colonic biopsies. […] We recommend that patients with suspected PSC undergoing ERCP should receive prophylactic antibiotics. […] We recommend that non-invasive investigations such as MRCP, dynamic liver MRI and/or contrast CT should be performed in patients who have new or changing symptoms or evolving abnormalities in laboratory investigations.
#65 Primary Sclerosing Cholangitis (PSC) – Hepatic and Biliary Disorders – Merck Manual Professional Edition
https://www.merckmanuals.com/professional/hepatic-and-biliary-disorders/gallbladder-and-bile-duct-disorders/primary-sclerosing-cholangitis-psc
Primary sclerosing cholangitis (PSC) is suspected in patients with unexplained abnormalities in liver tests, particularly in those with inflammatory bowel disease. A cholestatic pattern is typical: elevated alkaline phosphatase and gamma-glutamyltransferase (GGT) rather than aminotransferases. […] Diagnosis requires cholangiography to show multiple strictures and dilations in the intrahepatic and extrahepatic bile ducts. Cholangiography should begin with magnetic resonance cholangiopancreatography (MRCP). Endoscopic retrograde cholangiopancreatography (ERCP) is usually a 2nd choice because it is invasive. […] Adults with PSC, even in the absence of cirrhosis, should undergo abdominal imaging (ultrasound, abdominal computed tomography, or magnetic resonance imaging/magnetic resonance cholangiopancreatography) every 6 to 12 months to screen for gallbladder cancer and cholangiocarcinoma. […] Colonoscopy with biopsies should be done in patients without pre-existing inflammatory bowel disease (IBD) at the time of diagnosis of PSC and should be carried out annually in patients with PSC and IBD from the time of diagnosis of PSC due to the increased risk of colorectal adenocarcinoma.
#66 British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis | Gut
https://gut.bmj.com/content/68/8/1356
We recommend that where cholangiocarcinoma is suspected, contrast-enhanced, cross-sectional imaging remains the initial preferred investigation for diagnosis and staging. […] We recommend that patients with PSC who have coexistent colonic inflammatory bowel disease (IBD) should have annual colonoscopic surveillance from the time of diagnosis of colitis in line with the British Society of Gastroenterology (BSG) guidelines. […] We recommend that eligibility and referral should be assessed in line with the national guidelines.
#67 :: KJR :: Korean Journal of Radiology
https://www.kjronline.org/DOIx.php?id=10.3348/kjr.2016.17.1.25
The typical MR cholangiographic features include diffuse, multifocal short segmental strictures and mild dilatation in the intrahepatic and extrahepatic bile ducts alternating with normal ducts, which sometimes produce „beaded” appearance. […] As the fibrosis progresses and strictures worsen, the peripheral bile ducts are obliterated and become poorly visualized on MR cholangiography showing a „pruned tree” appearance. […] The clinical presentation can vary, including cholestatic laboratory findings and nonspecific symptoms including right upper quadrant pain or jaundice. […] A liver biopsy is not recommended for the diagnosis of PSC in patients with typical cholangiographic findings. […] Patients with PSC have a 10 to 15% risk of developing cholangiocarcinomas, and a 7 to 9% chance of a 10-year cumulative incidence of cholangiocarcinomas. […] Diagnostic evaluation of suspected cholangiocarcinoma includes serum cancer antigen 19-9, imaging studies such as MR, CT and endoscopic cholangiography with brushing or cytology. […] In patients with advanced liver disease, liver transplantation is recommended as a curative treatment option.
#68 Diagnosis and management of primary sclerosing cholangitis-perspectives from a therapeutic endoscopist
https://www.wjgnet.com/1948-5182/full/v7/i5/799.htm
High bilirubin level, presence of a common bile duct stricture and any dominant stricture have been found to be predictors of successful outcomes with clinical and laboratory improvement after endoscopic retrograde cholangiopancreaticography (ERCP). […] Endoscopic balloon dilatation with or without stenting have been used. […] The risk of developing CCA in PSC patients after 10 years and 20 years is 9% and 19% respectively. Thus a therapeutic endoscopist plays a key role in screening/surveillance for CCA in patients with PSC who have dominant strictures. […] A cut off value of cancer antigen 19-9 (CA19-9) of 130 U/mL in symptomatic patients has a sensitivity and specificity of 79% and 98% respectively. […] Studies on the utility of bile duct brushings in the diagnosis of CCA have reported a wide range of results. A meta-analysis performed by us found a high specificity of 97% but only a modest sensitivity of 43% across 54 studies.
#69 British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis | Gut
https://gut.bmj.com/content/68/8/1356
We recommend that patients with PSC should ordinarily not undergo ERCP until there has been expert multidisciplinary assessment to justify endoscopic intervention. […] We recommend that in patients undergoing ERCP for dominant strictures, pathological sampling of suspicious strictures is mandatory. […] We recommend that in patients undergoing ERCP for dominant strictures, biliary dilatation is preferred to the insertion of biliary stents. […] We recommend that patients with symptomatic, evolving or complex disease should be referred for expert multidisciplinary assessment. […] We recommend that all patients with PSC should have a risk assessment for osteoporosis. […] We recommend that in patients with fatigue, alternative causes should be actively sought and treated. […] We recommend that when a diagnosis of cholangiocarcinoma is clinically suspected, referral for specialist multidisciplinary meeting (MDM) review is essential.
#70 Primary sclerosing cholangitis – Wikipedia
https://en.wikipedia.org/wiki/Primary_sclerosing_cholangitis
Most people with PSC have evidence of autoantibodies and abnormal immunoglobulin levels. For example, approximately 80% of people with PSC have perinuclear antineutrophil cytoplasmic antibodies (P-ANCA); however, this and other immunoglobulin findings are not specific to those with PSC and are of unclear clinical significance/consequence. […] The differential diagnosis can include primary biliary cholangitis (formerly referred to as primary biliary cirrhosis), drug-induced cholestasis, cholangiocarcinoma, IgG4-related disease, post-liver transplantation nonanastomotic biliary strictures, and HIV-associated cholangiopathy. […] Primary sclerosing cholangitis and primary biliary cholangitis are distinct entities and exhibit important differences, including the site of tissue damage within the liver, associations with IBD, which includes ulcerative colitis and Crohn’s disease, response to treatment, and risks of disease progression.
#71 Sclerosing Cholangitis | Houston, Texas USA |
https://www.texasliver.com/sclerosing-cholangitis/
Most patients with PSC are diagnosed by discovering elevated serum alkaline phosphatase and gammaglutamyltranspeptidase activities on biochemical testing for other reasons (e. g. during evaluation of inflammatory bowel disease). […] The diagnosis of PSC is made by endoscopic retrograde cholangiopancreatography (ERCP). The characteristic findings include multifocal strictures and dilatations of the intrahepatic and extrahepatic bile ducts. […] Secondary causes of sclerosing cholangitis must be ruled out when making the diagnosis of PSC. Causes of secondary sclerosing cholangitis include drugs, bile duct cancers, past biliary tree surgery and opportunistic infections of the bile ducts that can cause a similar picture in patients with AIDS. These causes of secondary sclerosing cholangitis can usually be ruled-out by history, physical examination and laboratory tests.
#72 Sclerosing Cholangitis | Houston, Texas USA |
https://www.texasliver.com/sclerosing-cholangitis/
Most patients with PSC are diagnosed by discovering elevated serum alkaline phosphatase and gammaglutamyltranspeptidase activities on biochemical testing for other reasons (e. g. during evaluation of inflammatory bowel disease). […] The diagnosis of PSC is made by endoscopic retrograde cholangiopancreatography (ERCP). The characteristic findings include multifocal strictures and dilatations of the intrahepatic and extrahepatic bile ducts. […] Secondary causes of sclerosing cholangitis must be ruled out when making the diagnosis of PSC. Causes of secondary sclerosing cholangitis include drugs, bile duct cancers, past biliary tree surgery and opportunistic infections of the bile ducts that can cause a similar picture in patients with AIDS. These causes of secondary sclerosing cholangitis can usually be ruled-out by history, physical examination and laboratory tests.
#73 Sclerosing Cholangitis | Houston, Texas USA |
https://www.texasliver.com/sclerosing-cholangitis/
Most patients with PSC are diagnosed by discovering elevated serum alkaline phosphatase and gammaglutamyltranspeptidase activities on biochemical testing for other reasons (e. g. during evaluation of inflammatory bowel disease). […] The diagnosis of PSC is made by endoscopic retrograde cholangiopancreatography (ERCP). The characteristic findings include multifocal strictures and dilatations of the intrahepatic and extrahepatic bile ducts. […] Secondary causes of sclerosing cholangitis must be ruled out when making the diagnosis of PSC. Causes of secondary sclerosing cholangitis include drugs, bile duct cancers, past biliary tree surgery and opportunistic infections of the bile ducts that can cause a similar picture in patients with AIDS. These causes of secondary sclerosing cholangitis can usually be ruled-out by history, physical examination and laboratory tests.
#74 Pathology Pearls Post 8: Primary Sclerosing Cholangitis (PSC) | AASLD
https://www.aasld.org/liver-fellow-network/core-series/pathology-pearls/pathology-pearls-post-8-primary-sclerosing
Given the absence of etiology for secondary cholangitis such as infection and neoplasia, the histologic findings are suggestive of primary sclerosing cholangitis (PSC) […] Although the gold standard for the diagnosis of PSC remains endoscopic retrograde cholangiopancreatography (ERCP) / magnetic resonance cholangiopancreatography (MRCP), a liver biopsy can aid in the diagnosis. […] The most classic findings of PSC include large bile ducts surrounded by concentric fibrosis, which is also referred to as onion skin fibrosis. […] When affected large bile ducts are not sampled, findings in liver biopsies from patients with PSC may be nonspecific and can demonstrate portal tracts with increased inflammation and fibrosis. […] Primary biliary cholangitis (PBC) should also be considered in the differential diagnosis of PSC. […] Although less common, IgG4-associated cholangitis can mimic PSC.
#75 Sclerosing Cholangitis | Houston, Texas USA |
https://www.texasliver.com/sclerosing-cholangitis/
Most patients with PSC are diagnosed by discovering elevated serum alkaline phosphatase and gammaglutamyltranspeptidase activities on biochemical testing for other reasons (e. g. during evaluation of inflammatory bowel disease). […] The diagnosis of PSC is made by endoscopic retrograde cholangiopancreatography (ERCP). The characteristic findings include multifocal strictures and dilatations of the intrahepatic and extrahepatic bile ducts. […] Secondary causes of sclerosing cholangitis must be ruled out when making the diagnosis of PSC. Causes of secondary sclerosing cholangitis include drugs, bile duct cancers, past biliary tree surgery and opportunistic infections of the bile ducts that can cause a similar picture in patients with AIDS. These causes of secondary sclerosing cholangitis can usually be ruled-out by history, physical examination and laboratory tests.
#76 Pathology Pearls Post 8: Primary Sclerosing Cholangitis (PSC) | AASLD
https://www.aasld.org/liver-fellow-network/core-series/pathology-pearls/pathology-pearls-post-8-primary-sclerosing
Given the absence of etiology for secondary cholangitis such as infection and neoplasia, the histologic findings are suggestive of primary sclerosing cholangitis (PSC) […] Although the gold standard for the diagnosis of PSC remains endoscopic retrograde cholangiopancreatography (ERCP) / magnetic resonance cholangiopancreatography (MRCP), a liver biopsy can aid in the diagnosis. […] The most classic findings of PSC include large bile ducts surrounded by concentric fibrosis, which is also referred to as onion skin fibrosis. […] When affected large bile ducts are not sampled, findings in liver biopsies from patients with PSC may be nonspecific and can demonstrate portal tracts with increased inflammation and fibrosis. […] Primary biliary cholangitis (PBC) should also be considered in the differential diagnosis of PSC. […] Although less common, IgG4-associated cholangitis can mimic PSC.
#77 Sclerosing Cholangitis | Houston, Texas USA |
https://www.texasliver.com/sclerosing-cholangitis/
Most patients with PSC are diagnosed by discovering elevated serum alkaline phosphatase and gammaglutamyltranspeptidase activities on biochemical testing for other reasons (e. g. during evaluation of inflammatory bowel disease). […] The diagnosis of PSC is made by endoscopic retrograde cholangiopancreatography (ERCP). The characteristic findings include multifocal strictures and dilatations of the intrahepatic and extrahepatic bile ducts. […] Secondary causes of sclerosing cholangitis must be ruled out when making the diagnosis of PSC. Causes of secondary sclerosing cholangitis include drugs, bile duct cancers, past biliary tree surgery and opportunistic infections of the bile ducts that can cause a similar picture in patients with AIDS. These causes of secondary sclerosing cholangitis can usually be ruled-out by history, physical examination and laboratory tests.
#78 Primary Sclerosing Cholangitis â PSC | Choose the Right Test
https://arupconsult.com/content/primary-sclerosing-cholangitis-psc
Because antimitochondrial antibodies (AMAs) are a hallmark of PBC but are absent in PSC, AMA testing may be useful to differentiate between the two ALDs. […] Although there are no established prognostic tools for PSC, a patients biochemical profile at the time of diagnosis may inform prognosis. […] An increased serum immunoglobulin G4 (IgG4) may correlate with a more severe disease course. […] The most commonly used model is the revised Mayo natural history model for PSC, which uses age, bilirubin level, albumin level, AST level, and history of variceal bleeding to predict a patients probability of survival. […] Regular screening should be performed for other disorders commonly associated with PSC. […] Because of the high prevalence of IBD in patients with PSC, patients should undergo a full colonoscopy with biopsies at the time of diagnosis to screen for associated IBD.
#79 Primary Sclerosing Cholangitis: A Clinical Update – European Medical Journal
https://www.emjreviews.com/hepatology/article/primary-sclerosing-cholangitis-a-clinical-update/
Liver biopsy is performed to rule out other diseases, to look for AIH overlap, or in cases of suspected small duct PSC. […] Various classifications have been used to describe the cholangiographic findings in PSC. […] Dominant strictures have been defined in patients with PSC as stenosis of 1.5 mm in the common bile duct and 1.0 mm in the hepatic duct within 2 cm from the main hepatic confluence. […] Risk of malignancy in one cohort with dominant strictures was 6%. […] The estimated lifetime risk of CCA is 83-86% in patients with PSC. […] MRI can be used for diagnosis of CCA in PSC. […] Yearly carbohydrate antigen 19-9 levels tests and MRI are suggested for the surveillance of CCA in patients with PSC. […] The most commonly used risk score is the revised Mayo Score, which includes age, albumin, bilirubin, variceal bleeding, and AST levels.
#80 Primary Sclerosing Cholangitis â PSC | Choose the Right Test
https://arupconsult.com/content/primary-sclerosing-cholangitis-psc
Because antimitochondrial antibodies (AMAs) are a hallmark of PBC but are absent in PSC, AMA testing may be useful to differentiate between the two ALDs. […] Although there are no established prognostic tools for PSC, a patients biochemical profile at the time of diagnosis may inform prognosis. […] An increased serum immunoglobulin G4 (IgG4) may correlate with a more severe disease course. […] The most commonly used model is the revised Mayo natural history model for PSC, which uses age, bilirubin level, albumin level, AST level, and history of variceal bleeding to predict a patients probability of survival. […] Regular screening should be performed for other disorders commonly associated with PSC. […] Because of the high prevalence of IBD in patients with PSC, patients should undergo a full colonoscopy with biopsies at the time of diagnosis to screen for associated IBD.
#81 Primary Sclerosing Cholangitis: A Clinical Update – European Medical Journal
https://www.emjreviews.com/hepatology/article/primary-sclerosing-cholangitis-a-clinical-update/
Liver biopsy is performed to rule out other diseases, to look for AIH overlap, or in cases of suspected small duct PSC. […] Various classifications have been used to describe the cholangiographic findings in PSC. […] Dominant strictures have been defined in patients with PSC as stenosis of 1.5 mm in the common bile duct and 1.0 mm in the hepatic duct within 2 cm from the main hepatic confluence. […] Risk of malignancy in one cohort with dominant strictures was 6%. […] The estimated lifetime risk of CCA is 83-86% in patients with PSC. […] MRI can be used for diagnosis of CCA in PSC. […] Yearly carbohydrate antigen 19-9 levels tests and MRI are suggested for the surveillance of CCA in patients with PSC. […] The most commonly used risk score is the revised Mayo Score, which includes age, albumin, bilirubin, variceal bleeding, and AST levels.
#82 Primary sclerosing cholangitis in adults: Clinical manifestations and diagnosis – UpToDate
https://www.uptodate.com/contents/primary-sclerosing-cholangitis-in-adults-clinical-manifestations-and-diagnosis
Primary sclerosing cholangitis (PSC) is a chronic progressive disorder of unknown etiology that is characterized by inflammation, fibrosis, and stricturing of medium and large ducts in the intrahepatic and/or extrahepatic biliary tree. […] This topic will review the clinical manifestations and diagnosis of PSC. […] Our approach is largely consistent with the American Association for the Study of Liver Diseases, the European Association for the Study of Liver, and the American College of Gastroenterology. […] The discussion that follows is generally consistent with these guidelines. […] The diagnosis of PSC involves a general diagnostic approach, cholangiography, liver biopsy, and transient elastography.
#83 Primary Sclerosing Cholangitis
https://austinpublishinggroup.com/radiology/fulltext/ajr-v3-id1057.php
The diagnosis is very important because the risk of colorectal malignancies and cholangiocarcinoma is very high in these patients. […] Early diagnosis and management is essential to delay the progress of the disease. […] Primary sclerosing cholangitis is a chronic liver disease of unknown etiology, characterised primarily by inflammation and fibrosis of the biliary tree. […] Diagnosis is achieved by imaging, laboratory markers and histology. […] ERCP is the current gold standard investigation, although MRCP is the non-invasive investigation of choice.
#84 Understanding Primary Sclerosing Cholangitis Radiology
https://drniveditapandey.com/cholangitis/primary-sclerosing-cholangitis-radiology/
By visualizing these abnormalities, imaging techniques aid in confirming the diagnosis of PSC. […] Overall, the clinical presentation of primary sclerosing cholangitis can vary, but a combination of clinical symptoms, liver function tests, autoantibodies, and imaging techniques is essential for an accurate diagnosis. […] When it comes to diagnosing and monitoring primary sclerosing cholangitis (PSC), various imaging techniques play a crucial role. […] These imaging techniques, along with clinical symptoms and other diagnostic tests, help healthcare professionals accurately diagnose primary sclerosing cholangitis and monitor disease progression. […] Ultrasound, CT, MRI, and MR cholangiography are valuable imaging modalities that play a crucial role in detecting changes in the liver and bile ducts, aiding in the diagnosis of PSC. […] The diagnosis of PSC relies on classical imaging features in the correct clinical context and the exclusion of secondary causes of cholangitis.