AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Differentials
• Radiograph
• CT SCAN
• MRI
• Ultrasound
• Nuclear Medicine
• Intervention
• Multimedia
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Differentials
• Radiograph
• CT SCAN
• MRI
• Ultrasound
• Nuclear Medicine
• Intervention
• Multimedia
• References

Background

Primary sclerosing cholangitis (PSC) is a chronic, progressive, inflammatory disease characterized by fibrosis of the bile ducts. The cause is unknown, but a hypersensitivity reaction is implicated. Patients present with abnormalities of liver function tests and progressive intermittent obstructive jaundice, which may be associated with fever chills, night sweats, pain, and itching. A liver biopsy, endoscopic retrograde cholangiopancreatogram (ERCP), or percutaneous cholangiogram can help achieve diagnosis. The term primary is used to distinguish this condition from bile duct strictures that are secondary to bile duct injury, cholelithiasis, ischemia, and chemical injury.^{1, 2, 3, 4, 5}

See also the following topics in eMedicine:
Cholangitis [Emergency Medicine]
Cholangitis [Gastroenterology]
Primary Sclerosing Cholangitis [Gastroenterology]

See also the following topics in Medscape:
Resource Center Inflammatory Bowel Disease
Resource Center Gallbladder and Biliary Disease
Resource Center Liver & Intestine Transplant
Resource Center Pancreatitis
CME Inflammatory Bowel Disease
CME Therapy for Inflammatory Bowel Disease

Pathophysiology

PSC is a progressive inflammatory disease causing multifocal strictures of both the intrahepatic and extrahepatic bile ducts. The etiology is unknown, but a hypersensitivity reaction is speculated. PSC is often associated with inflammatory bowel disease, such as ulcerative colitis (50-74%) and Crohn disease (13%).⁶ This may account for the genetic predilection. Other associations include cirrhosis, chronic active hepatitis, pericholangitis, steatosis of the liver, pancreatitis, retroperitoneal and mediastinal fibrosis, Peyronie disease, Riedel thyroiditis, hypothyroidism, and retro-orbital pseudotumor.¹

The distribution of biliary system involvement varies, but the common bile duct (CBD) is almost always involved, with or without involvement of the intrahepatic bile ducts and, in some patients, the gallbladder and pancreas. Intrahepatic pigment stones are present in 8% of patients.⁶ 

Histologically, the disease can be classified into 4 stages.

• Stage 1: Histologic analysis in stage 1 disease identifies degeneration of the epithelial cells lining the bile ducts associated with inflammatory cell ductal and periportal triad infiltration and scarring.
• Stage 2: Stage 2 disease is characterized by fibrosis, paucity of bile ductules, periportal inflammatory cell infiltration, and piecemeal necrosis of the periportal hepatocytes.
• Stage 3: In stage 3 disease, severe degenerative changes are associated with disappearance of the bile ducts, portal-to-portal fibrous septa, and periportal cholestasis.
• Stage 4: Stage 4 depicts end-stage disease characterized by secondary biliary cirrhosis.

Frequency

United States

Prevalence of the disease in the general population is 1%, as compared with 4% in patients with inflammatory bowel disease.^{6, 7}

Mortality/Morbidity

The course of PSC is prolonged, with progressive jaundice. Death eventually ensues as a result of secondary biliary cirrhosis. The mainstay of treatment remains unsatisfactory, with conventional surgery rarely contemplated and steroid therapy of little use. Avoid surgical procedures, because they may complicate or even preclude future liver transplantation. Prolonged biliary drainage and bile duct dilatation by percutaneous transhepatic biliary stenting or ERCP may provide relief of symptoms. Over the past decade, liver transplantation has emerged as the treatment of choice for patients with end-stage primary sclerosing cholangitis, with many centers reporting 1-year patient and graft survival of 90-97% and 85-88%, respectively. However, specific complications to primary sclerosing cholangitis remain a problem.^{8, 9}

Inflammatory bowel disease complicates approximately 75% of the patients with primary sclerosing cholangitis, with an increased risk of large bowel cancer both pretransplantation and posttransplantation. Furthermore, symptoms from inflammatory bowel disease may become more severe following transplantation prompting proctocolectomy. The results of liver transplantation in patients with primary sclerosing cholangitis complicated by cholangiocarcinoma are disappointing. However, patients who are found to have an incidental cholangiocarcinoma have a low incidence of recurrence. The incidence of both acute and chronic rejection, hepatic artery thrombosis, and subsequent bile duct strictures appears to be higher in patients undergoing liver transplantation for primary sclerosing cholangitis. At least 20% of patients undergoing liver transplantation develop recurrentprimarysclerosingcholangitiswithin5 years.⁸

See also the following related topics in Medscape:
Resource Center Inflammatory Bowel Disease
Resource Center Colorectal Cancer

Race

No racial predilection exists, although the Japanese have reported 2 age peaks in their population at 20-30 years and 50-70 years.^{7, 10}

Sex

Male-to-female ratio is 7:3.⁶

Age

Most patients are younger than 45 years, with an age range of 21-67 years.⁶

Anatomy

The right and left hepatic ducts unite at the porta hepatis to form the common hepatic duct (CHD), which enters the free edge of the lesser omentum. The CHD is joined by the cystic duct, forming the CBD. The main left and right bile ducts lie anterior to the left and right portal veins. The hepatic artery branch usually lies anterior to the right portal vein but is often posterior to the left portal vein. In the liver, the bile ducts run with the portal vein and hepatic artery branches in a common sheath (ie, the portal triad).

The CBD is approximately 8 cm long. It lies in the free edge of the lesser omentum, usually situated anterolateral to the portal vein. It then passes behind the superior part of the second part of duodenum and traverses the head of the pancreas to the end at the duodenal papilla. Behind the duodenum, the CBD lies anterior to the portal vein with the gastroduodenal artery on its left side. Behind the head of the pancreas, the CBD lies on the inferior vena cava. At this point, the CBD receives the common pancreatic duct and turns to the right to enter the duodenum. Just below the porta hepatis, the right branch of the hepatic artery is usually visible passing between the CHD anteriorly and the portal vein posteriorly.

The upper limit of the normal diameter for the CBD is 5 mm; 6-7 mm is regarded as equivocal, and greater than 7 mm usually is pathologic, although bile duct diameter increases in elderly persons and post cholecystectomy. The normal bile duct is 4 mm or less in diameter in 95% of the adult population. In 98% of the adult population, the CHD is 5 mm or less in diameter at the porta hepatis, while at the head of the pancreas, the CBD normally shows slight narrowing. The intrahepatic duct just proximal to the CHD measures 2-3 mm in diameter. The cystic duct lies posterior to the CBD in 95% of the population and anterior to the CBD in 5%. The cystic duct runs in a common sheath with the CBD before their lumina unite; thus, cholecystectomy leaves a variable length of cystic duct in situ.

The CBD is distensible and responsive to fluctuation in prandial bile flow. It enlarges slowly with age at a pace equal to that of the hepatic artery and equal to half that of the portal vein. The duct is slightly larger in children with contracted gallbladder, in contrast to findings in adults. Often, a discrepancy exists between the CBD diameter measurements demonstrated using ERCP and ultrasound (US). This finding has been attributed to the variable cross section of the CBD, which is oval in 70% of patients.

Standard US technique involves measuring the anteroposterior (AP) diameter of the CBD. The AP diameter for oval and round ducts is similar, but the transverse diameter of an oval duct is greater. Thus, a discrepancy is found between ERCP and US findings when AP diameters are measured on US and conventionally transverse diameters are measured on ERCP. In addition, ERCP involves the injection of contrast, during which the biliary system is stretched by the pressure of injection. Since transverse CBD measurements using US correlate better with disease than conventional AP diameters, transverse measurements can be useful in confirming or excluding bile duct dilatation when AP diameters are larger than normal.

Postcholecystectomy CHD and CBD dimensions usually are slightly greater than they are prior to surgery. The normal postcholecystectomy CHD mean diameter is 0.52 cm at the porta hepatis and 0.62 cm at the CBD. Patients with gallstones also tend to have larger extrahepatic bile ducts, although this does not always mean that gallstones are noted within the bile ducts at the same examination.

See also the following related topics in eMedicine:
Gallbladder Disease
Bedside Ultrasonography, Gallbladder Disease
Bladder Stones


See also the following related topics in Medscape:
Resource Center Gallbladder and Biliary Disease
Resource Center Stone Disease

Clinical Details

Initially, patients come under observation because of abnormal liver function test results demonstrating raised alkaline phosphatase, gamma-glutamyltransferase, and mildly elevated bilirubin levels. All patients eventually present with chronic obstructive jaundice. Patients with known inflammatory bowel disease may present with raised liver enzymes before the onset of jaundice. Of patients with PSC, 10-15% may present with fever, night sweats, chills, itching, and right upper quadrant pain. In 53% of patients, a history of previous biliary surgery and/or recurrent pancreatitis also is found.⁶

Preferred Examination

US is the initial examination of choice in patients presenting with jaundice and right upper quadrant pain. The liver may demonstrate nonspecific abnormalities on US, which infrequently leads to definitive diagnosis. The primary role of US is in helping clinicians make the diagnosis of other bile duct mechanical obstructions, such as gallstones and neoplasia.¹¹

ERCP and percutaneous transhepatic cholangiography (PTC) remain the preferred investigations. Advances in software have improved the diagnostic quality of magnetic resonance cholangiopancreatography (MRCP),¹² and although preliminary studies indicate that MRCP may be sensitive and specific in PSC, larger studies are required before it replaces traditional invasive methods.^{11, 13, 14, 15, 16, 17, 18}

Limitations of Techniques

Both ERCP and PTC are invasive and can potentially cause ascending cholangitis. Recent technical advances have improved MRCP such that third- and fourth-order intrahepatic bile ducts can be resolved, but appearances remain nonspecific.

CT scans also demonstrate nonspecific changes. Indistinguishable features may occur with cholangiocarcinoma and other forms of cholangitis.

DIFFERENTIALS

Section 3 of 11  

• Authors and Editors
• Introduction
• Differentials
• Radiograph
• CT SCAN
• MRI
• Ultrasound
• Nuclear Medicine
• Intervention
• Multimedia
• References

Other Problems to Be Considered

Sclerosing cholangiocarcinoma
Acute ascending cholangitis
Primary biliary cirrhosis
AIDS-related cholangiopathy

RADIOGRAPH

Section 4 of 11  

• Authors and Editors
• Introduction
• Differentials
• Radiograph
• CT SCAN
• MRI
• Ultrasound
• Nuclear Medicine
• Intervention
• Multimedia
• References

Findings

Plain radiographs have little role to play in the diagnosis of PSC. Cholangiography in PSC is performed using either PTC or ERCP. ERCP is preferable because of its higher success rate in the absence of dilated intrahepatic bile ducts.

• Cholangiographic features of PSC include predominantly intrahepatic ductal disease with short multiple strictures associated with multifocal mild ductal dilations.
• Cholangiography demonstrates a pruned-tree appearance with filling of the central ducts and diffuse obstruction of the peripheral smaller radicles.
• More advanced disease is associated with long strictures.
• Bile duct dilations may result from the inflammatory process or distal obstruction.
• Multifocal strictures have a predilection for bifurcations.
• Skip lesions may be observed in which the duct is of normal caliber.
• The above features provide a beaded appearance to the ducts.
• Coarse nodularity with mural irregularities may produce a cobblestone appearance.
• Ductal diverticula or pseudodiverticula observed as small saccular outpouchings are pathognomonic findings for PSC.
• The CBD is almost always involved.
• The cystic duct also shows strictures, mural nodularity, and dilations with diverticula formation in 18% of patients.
• Gallbladder irregularity is uncommon.
• Pancreatic duct strictures and irregularities are observed in 8% of patients.⁶
• Intrahepatic pigment calculi, which are easily crushable, are found in 8% of patients and may cause mechanical obstruction. Rarely, these calculi may obstruct the CBD; however, bile duct dilatation may not occur in the presence of cholangiocarcinoma.⁶

Degree of Confidence

Appearances of the terminal bile ducts vary, particularly on ERCP, and overinterpretation is common. To tackle this problem, numerous views are obtained at various stages of contrast filling, which may help resolve equivocal changes. Radiographic distinction between a cholangiocarcinoma and sclerosing cholangitis occasionally may be impossible, because the diagnostic specificity of intrahepatic bile duct changes remains controversial.

False Positives/Negatives

Ascending cholangitis, AIDS-related cholangiopathy, cholangiocarcinoma, chemotherapy-induced cholangitis, eosinophilic cholangitis, recurrent pyogenic cholangitis, cholangitis secondary to parasites, and primary biliary cirrhosis can produce similar radiographic appearances.

CT SCAN

Section 5 of 11  

• Authors and Editors
• Introduction
• Differentials
• Radiograph
• CT SCAN
• MRI
• Ultrasound
• Nuclear Medicine
• Intervention
• Multimedia
• References

Findings

• Intrahepatic bile duct changes demonstrated on CT scans reflect cholangiography features with pruning and beading of the ducts. Pruning on CT scans is defined as the presence on a single CT slice of a 4-cm or longer segment of dilated duct (excluding the main right and left ducts) that lacks the expected side branching. Beading is defined as at least 3 closely alternating regions of change in caliber in an intrahepatic duct on a single CT slice.
• Skip dilatations, defined as isolated dilated peripheral bile ducts with no visible connection to the other dilated ducts on contiguous images, are strongly suggestive of PSC.
• CT appearances of extrahepatic bile duct involvement by PSC include focal or diffuse eccentric or concentric involvement and wall thickening (>2 mm and <5 mm), bile duct dilatation, relative lack of dilatation proximal to an apparent bile duct stricture, and enhancing intraluminal intramural nodules (>1 cm in diameter) seen on thin-section high-resolution images.
• Contrast enhancement of the bile duct is a nonspecific finding that can be observed in both normal and abnormal bile ducts.
• As many as 65% of patients with PSC may have benign celiac, gastrohepatic ligament, porta hepatis, periaortic, pancreaticoduodenal, and mesenteric lymphadenopathy.^{19, 20}
• Lymph nodes are usually homogeneous and isodense with the pancreas.

Degree of Confidence

Intrahepatic bile duct changes on CT scans reflect cholangiographic features with pruning and beading of the ducts. Skip dilatations, defined as isolated dilatation of the intrahepatic bile ducts, are strongly suggestive of PSC; however, ductal delineation and demonstration of strictures can be difficult in the early stages using US or CT scans. CT has a complementary role to that of cholangiography; moreover, do not use CT as a screening examination.

False Positives/Negatives

Beading, pruning, irregularity, and asymmetry of the intrahepatic bile ducts as demonstrated on CT scans are not specific for PSC. Similar CT changes may be found in other forms of cholangitis and cholangiocarcinoma.

MRI

Section 6 of 11  

• Authors and Editors
• Introduction
• Differentials
• Radiograph
• CT SCAN
• MRI
• Ultrasound
• Nuclear Medicine
• Intervention
• Multimedia
• References

Findings

Biliary tract, liver parenchymal, and pancreatic features suggestive of PSC have been reported using MRI.^{21, 22}

• Use of T1-weighted fat-suppressed spin-echo pulse sequences, with or without intravenous gadolinium, facilitates visualization of the bile duct wall.
• Peripheral wedge-shaped areas of high T2-weighted signal intensity in the liver parenchyma and dilatation of the bile ducts are characteristic MRI features of PSC observed in approximately 72% of patients.
• Associated features include periportal edema (40%), lobar atrophy (8-28%), and portal hypertension (35%).
• Abnormal hyperintensity of the liver parenchyma on T1-weighted images may be observed in 23% of patients.
• Increased enhancement of the liver parenchyma on dynamic arterial-phase gradient-echo images, predominantly in the peripheral areas, may be observed in 56% of patients.
• Periportal inflammation may be demonstrated on MRI images as a region of low signal intensity on T1-weighted images and as intermediate signal between liver and bile on T2-weighted images.
• On gadolinium-enhanced images, enhancement of inflammatory periportal tissue permits distinction from nonenhancing periportal edema.
• Characteristic changes on MRI have been recognized within the pancreas, suggestive of PSC. Increased signal on T2-weighted images, decreased signal on T1-weighted images, enlargement of the pancreas, and decreased contrast enhancement are suggestive of pancreatic disease associated with PSC.
• MRCP may be used, but the extent of intrahepatic disease may be estimated incorrectly because ductal distension cannot be achieved using this technique.

Gadolinium-based contrast agents (gadopentetate dimeglumine [Magnevist], gadobenate dimeglumine [MultiHance], gadodiamide [Omniscan], gadoversetamide [OptiMARK], gadoteridol [ProHance]) have recently been linked to the development of nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD). For more information, see the eMedicine topic Nephrogenic Fibrosing Dermopathy. 

NSF/NFD has occurred in patients with moderate to end-stage renal disease after being given a gadolinium-based contrast agent to enhance MRI or MRA scans. As of late December 2006, the FDA had received reports of 90 such cases. Worldwide, over 200 cases have been reported, according to the FDA. NSF/NFD is a debilitating and sometimes fatal disease. Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellow spots on the whites of the eyes; joint stiffness with trouble moving or straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness. For more information, see the FDA Public Health Advisory or Medscape.

Degree of Confidence

The numbers of patients with PSC studied using MRI have been small; thus, the reliability of the changes described above is uncertain. MRCP may be used, but the extent of intrahepatic disease may be estimated incorrectly because ductal distention cannot be achieved using MRCP.

False Positives/Negatives

MRI features of PSC are not specific and may occur with cholangiocarcinoma and other forms of cholangitis.

ULTRASOUND

Section 7 of 11  

• Authors and Editors
• Introduction
• Differentials
• Radiograph
• CT SCAN
• MRI
• Ultrasound
• Nuclear Medicine
• Intervention
• Multimedia
• References

Findings

Sonographically, biliary abnormalities are not usually visible, unless biliary dilatation is associated.

• Intrahepatic saccular dilations occasionally may be visualized.
• In long-standing disease, echogenic intraductal structures, representing sludge or calculi, are visible on US images.
• Bile duct wall thickening and small intraluminal protrusions also have been reported in patients with PSC.
• In end-stage disease, US signs of portal hypertension may be demonstrated.
• Secondary biliary cirrhosis occurs as a part of the disease complex of PSC. Most patients with secondary biliary cirrhosis have no sonographic abnormalities, but occasionally, irregular segmental duct dilatation can be observed.
• Patients with advanced disease may show an increase in periportal echogenicity.
• Cirrhotic changes, such as nodularity-increased liver attenuation and splenomegaly, also may be apparent.

Degree of Confidence

US appearances of PSC are nonspecific; seldom can a confident diagnosis of PSC be made using US alone. The primary role of US is in the diagnosis of other causes of obstructive jaundice.

False Positives/Negatives

The differential diagnosis includes cholangiocarcinoma and other causes of ascending cholangitis and primary biliary cirrhosis. In addition to PSC, bile duct wall thickening is associated with hepatic clonorchiasis, Oriental cholangiohepatitis, biliary ascariasis, peribiliary cysts, and AIDS-related cholangiopathy.

NUCLEAR MEDICINE

Section 8 of 11  

• Authors and Editors
• Introduction
• Differentials
• Radiograph
• CT SCAN
• MRI
• Ultrasound
• Nuclear Medicine
• Intervention
• Multimedia
• References

Findings

Radionuclide scanning using technetium-99m (^99mTc) iminodiacetic acid compounds shows multiple focal areas of persistent activity distributed in the liver parenchyma. The clearance rate of the isotope through the liver is markedly prolonged, with gallbladder visualization achieved in 70% of patients.^{6, 23}

Degree of Confidence

Specificity of ^99mTc iminodiacetic acid scanning is low, but it is a valuable technique to use for quantifying biliary kinetics.

False Positives/Negatives

Prolonged isotope transit time may occur in other causes of hepatic dysfunction; similarly, nonvisualization of the gallbladder may occur with cystic duct obstruction and chronic gallbladder disease.

INTERVENTION

Section 9 of 11  

• Authors and Editors
• Introduction
• Differentials
• Radiograph
• CT SCAN
• MRI
• Ultrasound
• Nuclear Medicine
• Intervention
• Multimedia
• References

Patients with CBD strictures presenting with recurrent attacks of cholangitis can derive short-term benefit from endoscopic or percutaneous balloon dilatation. Many of these patients have pigment stone impaction at the site of the stricture. With successful placement of a guidewire across the stricture balloon, dilatation is usually straightforward. Although this results in the immediate relief of symptoms, the long-term benefit of stricture dilatation is unclear.^{24, 25, 26}

Medical/Legal Pitfalls

• Every effort should be made not to introduce infection with endoscopic manipulation. For similar reasons, sphincterotomy and the introduction of stents in strictures secondary to PSC should be used sparingly.

MULTIMEDIA

Section 10 of 11  

• Authors and Editors
• Introduction
• Differentials
• Radiograph
• CT SCAN
• MRI
• Ultrasound
• Nuclear Medicine
• Intervention
• Multimedia
• References

Media file 1:  Endoscopic retrograde cholangiopancreatography performed in a patient with abnormal liver function test results shows multiple intrahepatic bile duct strictures and beading.
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Media type:  X-RAY

Media file 2:  Double-contrast barium enema (same patient as Image 1) shows filiform polyps and an ahaustral colon resulting from ulcerative colitis.
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Media type:  X-RAY

Media file 3:  Percutaneous transhepatic cholangiogram shows dilatation, stricturing, and beading of the intrahepatic bile ducts. Note the surgical clips from a previous cholecystectomy.
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Media type:  X-RAY

Media file 4:  T-tube cholangiogram shows irregularity of the common bile duct, stricturing, beading, and dilatation of the intrahepatic bile ducts. Note a calculus in the termination of the left hepatic duct (arrow).
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Media type:  X-RAY

Media file 5:  Magnetic resonance cholangiopancreatography shows a normal-sized common bile duct, but strictures of both the left and right ducts are noted as well as a dilated proximal left hepatic duct.
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Media type:  MRI

Media file 6:  Technetium-99m iminodiacetic acid scan (same patient as Image 5) shows retention of the radionuclide proximal to strictures in the distribution of the left hepatic duct. Note the lack of filling of the gallbladder because of a previous cholecystectomy. Isotope has entered the small bowel.
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Media type:  Image

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Differentials
• Radiograph
• CT SCAN
• MRI
• Ultrasound
• Nuclear Medicine
• Intervention
• Multimedia
• References

1. Burak KW, Urbanski SJ, Swain MG. A case of coexisting primary biliary cirrhosis and primary sclerosing cholangitis: a new overlap of autoimmune liver diseases. Dig Dis Sci. Sep 2001;46(9):2043-7. [Medline].
2. Cameron RG, Blendis LM, Neuman MG. Accumulation of macrophages in primary sclerosing cholangitis. Clin Biochem. May 2001;34(3):195-201. [Medline].
3. Hawkes ND, Mutimer D, Thomas GA. Intermittent jaundice and rigors in a patient with longstanding ulcerative colitis. Postgrad Med J. Jun 2001;77(908):406-7, 412-3. [Medline].
4. Schrumpf E, Boberg KM. Primary sclerosing cholangitis: challenges of a new millenium. Dig Liver Dis. Dec 2000;32(9):753-5. [Medline].
5. Lazaridis KN. Dissecting the genetic susceptibility for cholangiocarcinoma in primary sclerosing cholangitis. Hepatology. Jan 2008;47(1):8-10. [Medline].
6. Dahnart W. Radiology Review Manual. 6th. Phildelphia: Lippincott William & Wilkins; 2007:699-700.
7. Takikawa H. Characteristics of primary sclerosing cholangitis in Japan. Hepatol Res. Oct 2007;37 Suppl 3:S470-3. [Medline].
8. Wiesner RH. Liver transplantation for primary sclerosing cholangitis: timing, outcome, impact of inflammatory bowel disease and recurrence of disease. Best Pract Res Clin Gastroenterol. Aug 2001;15(4):667-80. [Medline].
9. Freeman K, Shao Z, Remzi FH, Lopez R, Fazio VW, Shen B. Impact of orthotopic liver transplant for primary sclerosing cholangitis on chronic antibiotic refractory pouchitis. Clin Gastroenterol Hepatol. Jan 2008;6(1):62-8. [Medline].
10. Takagi I, Shibamoto Y, Toda G. [Primary sclerosing cholangitis (PSC)]. Ryoikibetsu Shokogun Shirizu. 2000;(31):226-9. [Medline].
11. Oikarinen H, Paakko E, Suramo I, et al. Imaging and estimation of the prognostic features of primary sclerosing cholangitis by ultrasonography and MR cholangiography. Acta Radiol. Jul 2001;42(4):403-8. [Medline].
12. Haider MA, Bret PM. The role of magnetic resonance cholangiography in primary sclerosing cholangitis. J Hepatol. Oct 2000;33(4):659-60. [Medline].
13. Bjornsson ES, Kilander AF, Olsson RG. Bile duct bacterial isolates in primary sclerosing cholangitis and certain other forms of cholestasis--a study of bile cultures from ERCP. Hepatogastroenterology. Nov-Dec 2000;47(36):1504-8. [Medline].
14. Goolamali SI, Taylor-Robinson SD. Endoscopic retrograde cholangiopancreatography appearances of early primary sclerosing cholangitis. J Clin Gastroenterol. May-Jun 2001;32(5):460-1. [Medline].
15. Keogan MT, Edelman RR. Technologic advances in abdominal MR imaging. Radiology. Aug 2001;220(2):310-20. [Medline].
16. Rajaram R, Ponsioen CY, Majoie CB, et al. Evaluation of a modified cholangiographic classification system for primary sclerosing cholangitis. Abdom Imaging. Jan-Feb 2001;26(1):43-7. [Medline].
17. van den Hazel SJ, Wolfhagen EH, van Buuren HR, et al. Prospective risk assessment of endoscopic retrograde cholangiography in patients with primary sclerosing cholangitis. Dutch PSC Study Group. Endoscopy. Oct 2000;32(10):779-82. [Medline].
18. Etzel JP, Eng SC, Ko CW, Lee SD, Saunders MD, Tung BY. Complications after ERCP in patients with primary sclerosing cholangitis. Gastrointest Endosc. Nov 30 2007;[Medline].
19. Hirche TO, Russler J, Braden B, Schuessler G, Zeuzem S, Wehrmann T. Sonographic detection of perihepatic lymphadenopathy is an indicator for primary sclerosing cholangitis in patients with inflammatory bowel disease. Int J Colorectal Dis. Nov 2004;19(6):586-94. [Medline].
20. Johnson KJ, Olliff JF, Olliff SP. The presence and significance of lymphadenopathy detected by CT in primary sclerosing cholangitis. Br J Radiol. Dec 1998;71(852):1279-82. [Medline].
21. Fulcher AS, Turner MA, Franklin KJ, et al. Primary sclerosing cholangitis: evaluation with MR cholangiography-a case-control study. Radiology. Apr 2000;215(1):71-80. [Medline].
22. Elsayes KM, Oliveira EP, Narra VR, Abou El Abbass HA, Ahmed MI, Tongdee R. MR and MRCP in the evaluation of primary sclerosing cholangitis: current applications and imaging findings. J Comput Assist Tomogr. May-Jun 2006;30(3):398-404. [Medline].
23. Jonas E, Hultcrantz R, Slezak P, et al. Dynamic 99Tcm-HIDA SPET: non-invasive measuring of intrahepatic bile flow. Description of the method and a study in primary sclerosing cholangitis. Nucl Med Commun. Feb 2001;22(2):127-34. [Medline].
24. Kaya M, Petersen BT, Angulo P, et al. Balloon dilation compared to stenting of dominant strictures in primary sclerosing cholangitis. Am J Gastroenterol. Apr 2001;96(4):1059-66. [Medline].
25. Linder S, Soderlund C. Endoscopic therapy in primary sclerosing cholangitis: outcome of treatment and risk of cancer. Hepatogastroenterology. Mar-Apr 2001;48(38):387-92. [Medline].
26. Lopez RR Jr, Cosenza CA, Lois J, et al. Long-term results of metallic stents for benign biliary strictures. Arch Surg. Jun 2001;136(6):664-9. [Medline].

Article Last Updated: Jan 4, 2008