Primary sclerosing cholangitis

Lee, Young-Mee; Kim, David J.

doi:10.1007/s11938-001-0012-8

Cited by 29 publications

(40 citation statements)

References 41 publications

(19 reference statements)

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“…The disease typically occurs in young men, 70% to 75% of whom have inflammatory bowel disease. 10 Direct radiographic visualization of the bile ducts by endoscopic, percutaneLiver Transplant Practice Guidelines ous, or operative cholangiography is required for accurate diagnosis.…”

Section: Pbcmentioning

confidence: 99%

Liver transplantation

Carithers

2000

Liver Transpl

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Liver transplantation has revolutionized the care of patients with end-stage liver disease. Liver transplantation is indicated for acute or chronic liver failure from any cause. Because there are no randomized controlled trials of liver transplantation versus no therapy, the efficacy of this surgery is best assessed by carefully comparing postoperative survival with the known natural history of the disease in question. The best examples of this are in primary biliary cirrhosis and primary sclerosing cholangitis, for which well-validated disease-specific models of natural history are available. There are currently relatively few absolute contraindications to liver transplantation. These include severe cardiopulmonary disease, uncontrolled systemic infection, extrahepatic malignancy, severe psychiatric or neurological disorders, and absence of a viable splanchnic venous inflow system. One of the most frequently encountered contraindications to transplantation is ongoing destructive behavior caused by drug and alcohol addiction. The timing of the surgery can have a profound impact on the mortality and morbidity of patients undergoing liver transplantation. Because of the long waiting lists for donor organs, the need to project far in advance when transplantation might be required has proven to be one of the greatest challenges to those treating patients with end-stage liver disease. Three important questions must be addressed in a patient being considered for liver transplantation: (1) when should the patient be referred for possible transplantation? (2) when should the patient be listed for transplantation? and (3) when is the patient too sick to have a reasonable chance of surviving the perioperative period?

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Section: Pbcmentioning

confidence: 99%

Liver transplantation

Carithers

2000

Liver Transpl

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“…1 The diagnosis of primary sclerosing cholangitis is based on characteristic cholangiographic findings, in combination with clinical, biochemical, and histological features. The disease lacks a definitive etiological factor, although a strong association with inflammatory bowel disease is well recognized.…”

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confidence: 99%

“…It must be noted that, patients with concomitant ulcerative colitis are at an increased risk for cholangiocarcinoma. 1 The disease typically does not respond to immunosuppressive therapy, and hepatic transplantation continues to be the only option to provide long-term survival benefit in these patients. 2 The term 'secondary sclerosing cholangitis' is used to designate cases in which a specific etiological factor is identified.…”

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confidence: 99%

IgG4-associated cholangitis: a comparative histological and immunophenotypic study with primary sclerosing cholangitis on liver biopsy material

et al. 2009

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IgG4-associated cholangitis is a steroid-responsive hepatobiliary inflammatory condition associated with autoimmune pancreatitis that clinically and radiologically mimics primary sclerosing cholangitis. In this study, we conducted a morphological and immunohistochemical analysis of liver material obtained from individuals with IgG4-associated cholangitis, and compared these with well-characterized cases of primary sclerosing cholangitis. The study group consisted of 10 patients (9 biopsy and 1 hepatectomy case) with IgG4-associated cholangitis and 17 patients with primary sclerosing cholangitis (16 needle biopsy and 1 hepatectomy case). All patients with IgG4-associated cholangitis had pancreatic involvement as well, and six pancreatectomy samples revealed characteristic histopathological features of autoimmune pancreatitis. Primary sclerosing cholangitis cases were defined by the presence of a characteristic ERCP appearance. Clinical, pathological, radiological, and follow-up data were recorded for all cases. Portal and periportal inflammation was graded according to Ishak's guidelines. Immunohistochemical stains for IgG and IgG4 were performed. The cohort of patients with IgG4-associated cholangitis (mean age: 63 years) was older than individuals with primary sclerosing cholangitis (mean age: 44 years). Seven of these cases showed intrahepatic biliary strictures. IgG4-associated cholangitis liver samples showed higher portal (P ¼ 0.06) and lobular (P ¼ 0.009) inflammatory scores. Microscopic portal-based fibro-inflammatory nodules that were composed of fibroblasts, plasma cells, lymphocytes, and eosinophils were exclusively observed in five of the IgG4-associated cholangitis cases (50%). More than 10 IgG4-positive plasma cells per HPF (high power field) were observed in 6 of the IgG4-associated cholangitis cases (mean: 60, range: 0-140 per HPF), whereas all primary sclerosing cholangitis cases showed significantly lesser numbers (mean: 0.08, range: 0-1 per HPF). On a liver biopsy, the histological features of IgG4-associated cholangitis may be distinctive, and in conjunction with IgG4 immunohistochemical stain, may help distinguish this disease from primary sclerosing cholangitis.

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“…The pathogenesis of PSC is unknown, but available data suggest that both immunological and nonimmunological host defenses may be impaired (1). Hence, the normal intestinal flora or their metabolic products may play a pathogenic role (1).…”

Section: Introductionmentioning

confidence: 99%

“…Hence, the normal intestinal flora or their metabolic products may play a pathogenic role (1). The disease is usually progressive and may lead to cirrhosis and portal hypertension.…”

Section: Introductionmentioning

confidence: 99%

Management of primary sclerosing cholangitis

Lee¹

2002

The American Journal of Gastroenterology

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PREAMBLEGuidelines for clinical practice are intended to indicate preferred approaches to medical problems as established by scientifically valid research. Double blind, placebo-controlled studies are preferable, but reports and expert review articles are also utilized in a thorough review of the literature conducted through the National Library of Medicine's MEDLINE. When only data that will not withstand objective scrutiny are available, a recommendation is identified as a consensus of experts. Guidelines are applicable to all physicians who address the subject, without regard to specialty training or interests, and are intended to indicate the preferable but not necessarily the only acceptable approach to a specific problem. Guidelines are intended to be flexible and must be distinguished from standards of care that are inflexible and rarely violated. Given the wide range of specifics in any health care problem, the physician must always choose the course best suited to the individual patient and the variables in existence at the moment of decision.Guidelines are developed under the auspices of the American College of Gastroenterology and its Practice Parameters Committee and approved by the Board of Trustees. Each has been intensely reviewed and revised by the Committee, other experts in the field, physicians who will use them, and specialists in the science of decision of analysis. The recommendations of each guideline are therefore considered valid at the time of their production based on the data available. New developments in medical research and practice pertinent to each guideline will be reviewed at an established time and indicated at publication to assure continued validity. (Am J Gastroenterol 2002;97:528 -534.

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Primary sclerosing cholangitis

Cited by 29 publications

References 41 publications

Liver transplantation

Liver transplantation

IgG4-associated cholangitis: a comparative histological and immunophenotypic study with primary sclerosing cholangitis on liver biopsy material

Management of primary sclerosing cholangitis

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