Beneficial effect of colchicine in a case of sclerosing cholangitis

A patient with ulcerative colitis and primary sclerosis cholangitis confirmed by liver biopsy and endoscopic retrograde cholangiography presented with severely altered liver function tests. A month of steroid treatment for the colitis symptoms had no effect on the liver function tests, and colchicine, 1 mg/day, was added. After 3 months of this combined treatment, improvement of the liver function tests was noted and progressed to complete normalization of all liver function tests at the end of a 2-year period of follow-up. On further follow-up, the patient remained asymptomatic and with normal liver function tests for an additional 3 years. Further controlled studies would be worthwhile to evaluate the possibility of treatment of primary sclerosis cholangitis with colchicine with or without steroids.     

A case of primary sclerosing cholangitis

A case of primary sclerosing cholangitis (PSC) is reported. A 16 year-old female developed right hypochondralgia and nausea without jaundice. Examination on admission showed elevation of SGOT, SGPT, Al-P, gamma-GTP and LAP activities, but T-Bil, AFP and CEA were within normal limits. Peripheral eosinocytes increased by 10%, and tests for HBsAg, antiHBs, antimitochondrial antibody and anti-smooth muscle antibody were all negative. ERCP revealed a narrowing of the proximal portion of the common the hepatic duct, and beading of the intrahepatic bile ducts. Liver scintigram and CT revealed no tumors in the liver, biliary tract or pancreas. Laparoscopy showed a smooth liver without swelling and a slightly swollen gallbladder. Histologically, the liver biopsy specimen showed ductal proliferation of small interlobular bile ducts and periductal fibrosis. No bile plugs, granuloma or distinct cholangitis were observed. No abnormal findings, including evidence of inflammatory bowel disease, were detected by barium enema. At present, one year after discharge, although her symptoms and liver function test abnormalities continue, she has been attending high school. Although 58 cases of PSC have been reported in Japan, juvenile cases occurring before the third decade number only 3 including ours.     

A case of segmental primary sclerosing cholangitis

A 74-year-old man was admitted to the Yokohama City University School of Medicine for investigation of high values of ALP and Y-GTP. Radiographic examinations, including abdominal computed tomography and percutaneous transhepatic cholangiography, strongly suggested bile duct cancer in the hepatic hilus. After left lobectomy, pathological examination disclosed segmental primary sclerosing cholangitis. Clinical examination cannot always distinguish primary sclerosing cholangitis from cancer. We report a case of segmental primary sclerosing cholangitis and discuss the diagnosis and the treatment of this disease.     

Long-standing asymptomatic primary sclerosing cholangitis report of three cases

Three patients, two males and one female, with asymptomatic primary sclerosing cholangitis (PSC) are described. The diagnosis was made in each case by endoscopic retrograde cholangiography after investigation of persistent elevation of the serum alkaline phosphatase. All three have remained completely well without any medical or surgical treatment for 3, 7, and 15 years, respectively, despite extensive involvement of the biliary tree. Follow-up liver biopsies in two have shown no histological evidence of progression to secondary biliary cirrhosis. PSC may occur more frequently and may follow a less severe clinical course than previously recognized.     

Update on primary sclerosing cholangitis

Early studies in primary sclerosing cholangitis (PSC) were concerned with disease characterization, and were followed by epidemiological studies of PSC and clinical subsets of PSC as well as a large number of treatment trials. Recently, the molecular pathogenesis and the practical handling of the patients have received increasing attention. In the present review we aim to give an update on the pathogenesis of PSC and cholangiocarcinoma in PSC, as well as to discuss the current opinion on diagnosis and treatment of PSC in light of the recent European Association for the Study of the Liver and the American Association for the Study of Liver Diseases practice guidelines.     

Pediatric primary sclerosing cholangitis

Summary An 11-year-old male presented with abdominal pain and emesis. Serum chemistries revealed cholestasis and an ERCP demonstrated sclerosing cholangitis. Secondary causes of this disease process were excluded. Sclerosing cholangitis is distinctly uncommon in the pediatric age group, but it should be considered in evaluation of patients of any age with cholestasis. It appears in some cases that there may be subtle histologic and radiographic features which separate the pediatric and adult forms of the disease.The opinions and assertions contained herein are the private ones of the authors and are not to be construed as official or reflecting the views of the Department of Defense, Department of the Navy or the Naval Service at large, or the Uniformed Services University of the Health Sciences.     

Small-duct primary sclerosing cholangitis

Current evidence suggests that PSC may affect the entire biliary system from interlobular bile ducts to the ampulla of Vater. If the affected bile ducts are too small for cholangiographic identification, the best designation for the condition is small-duct PSC; cholangiographically identifiable duct changes are the hallmark of large-duct PSC. The term "small-duct PSC" should replace the name pericholangitis because that designation lacks a clear definition. Small-duct PSC may occur in combination with large-duct PSC or it may occur alone. The clinical course of patients with PSC depends on the hepatic changes related to small-duct PSC, not primarily on the classic large-duct disease. Therefore the features of small-duct PSC and its complications are used to determine the disease stages of PSC. The incidence of small-duct PSC still is not known and the natural history as well as the pathogenesis of the condition remains obscure. Nevertheless, the role of small-duct disease in the syndrome of PSC has come into focus and the themes for future studies are apparent. Work should commence.     

Small-duct primary sclerosing cholangitis

Patients with cholestatic liver tests and typical histologic features of primary sclerosing cholangitis (PSC) but a normal cholangiogram have been identified as having small-duct PSC. This subgroup of PSC has been less well characterized than the classic largeduct form. Some patients characterized as having small-duct PSC develop cholangiographic features of PSC during follow-up. Three papers published in 2002 on small-duct PSC patients suggested a better prognosis in patients with small-duct PSC than in those with large-duct PSC. However, these studies included a limited number of patients and had a short follow-up. A combined cohort of these patients with a prolonged follow-up recently confirmed these previous observations. However, some patients will suffer from liver-related mortality or will need to undergo liver transplantation. Recurrence of small-duct PSC after liver transplantation has been reported. Cholangiocarcinoma, the most feared complication of PSC, has not been described in a patient with small-duct PSC without progression first to large-duct PSC.     

A case of primary sclerosing cholangitis mimicking chronic pancreatitis

Summary A case of a 37 year old male is presented, who died from primary sclerosing cholangitis (PSC) with characteristic radiological picture and laboratory data suggesting chronic pancreatitis (CP).     

A case of primary sclerosing cholangitis mimicking chronic pancreatitis

A case of a 37 year old male is presented, who died from primary sclerosing cholangitis (PSC) with characteristic radiological picture and laboratory data suggesting chronic pancreatitis (CP).     

Secondary sclerosing cholangitis: a comparison to primary sclerosing cholangitis

OBJECTIVES: The natural history of secondary sclerosing cholangitis (SSC) is ill-defined. In order to better determine the natural history of this condition, we retrospectively reviewed data from the Mayo Clinic in Rochester, Minnesota. We also compared the natural history of patients diagnosed with SSC to a cohort with a diagnosis of primary sclerosing cholangitis (PSC). METHODS: We used a computer-assisted search to identify patients with a diagnosis of SSC seen from 1992 to 2002. The diagnosis was confirmed by chart review and information about age, gender, etiology, therapy, and clinical course was sought. We excluded those presumed SSC patients who had a history of inflammatory bowel disease, those with malignancy at the time of diagnosis, and those who had undergone liver transplantation prior to the diagnosis of SSC. Patients with PSC matched for age, gender, and serum bilirubin level served as disease controls. RESULTS: We identified 31 patients, average age 57, (range 28-79). The causes of SSC included surgical trauma from cholecystectomy (13 patients), intraductal stones (12 patients), recurrent pancreatitis (4 patients), and abdominal injury (2 patients). Nine patients with SSC ultimately required liver transplantation and 4 patients have died. When compared to matched patients with PSC, the survival free of transplant was significantly shortened (p<0.03). CONCLUSIONS: When the long-term outcome of SSC patients was compared to matched PSC controls, the SSC patients had a poorer outcome. The natural history of SSC is characterized by a shortened life expectancy.     

Pathogenesis of primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) represents a chronic cholestatic liver disease with fibroobliterative sclerosis of intra- and/or extrahepatic bile ducts, eventually leading to biliary cirrhosis. The association with human leukocyte antigen (HLA) and non-HLA haplotypes and the presence of autoantibodies in sera of PSC patients support a crucial role for immune-mediated mechanisms in the initiation and progression of PSC. The strong clinical association between PSC and inflammatory bowel diseases led to intriguing pathogenetic concepts, in which the inflamed gut with translocation of bacterial products and homing of gut-primed memory T lymphocytes via aberrantly expressed adhesion molecules plays a fundamental role. Genetically or chemically modified bile composition was shown to induce sclerosing cholangitis and liver fibrosis in a number of animal models ("toxic bile concept"). The potential role of vascular injury with ischemia of bile duct epithelium cells in the development of sclerosing cholangitis is supported by animal models of endothelial cell injury showing close morphological similarities with human PSC.     

Management of primary sclerosing cholangitis

Guidelines 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.