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At least 19 recordsLinked to original sources
PubMed2024

Network proximity analysis as a theoretical model for identifying potential novel therapies in primary sclerosing cholangitis.

Primary Sclerosing Cholangitis (PSC) is a progressive cholestatic liver disease with no licensed therapies. Previous Genome Wide Association Studies (GWAS) have identified genes that correlate significantly with PSC, and these were identified by systematic review. Here we use novel Network Proximity Analysis (NPA) methods to identify already licensed candidate drugs that may have an effect on the genetically coded aspects of PSC pathophysiology.Over 2000 agents were identified as significantly linked to genes implicated in PSC by this method. The most significant results include previously researched agents such as metronidazole, as well as biological agents such as basiliximab, abatacept and belatacept. This in silico analysis could potentially serve as a basis for developing novel clinical trials in this rare disease.

Cholangitis, Sclerosing↗
PubMed1978

Primary sclerosing cholangitis.

Two new cases of primary sclerosing cholangitis with a rapid fatal course, particularly the patient in Case 2, are presented. Primary sclerosing cholangitis is a rare condition of unknown cause presenting with extrahepatic biliary obstruction due to a chronic inflammatory obliterative process with the absence of stones, cancer, or previous biliary surgery. This condition is often associated with ulcerative colitis, retroperitoneal fibrosis, and Riedel's thyroiditis. Surgical treatment for promoting bile drainage and long-term corticosteroid therapy are effective palliative measures.

Cholangitis↗
PubMedDate not supplied

Surgical problems in primary sclerosing cholangitis.

The characteristics of primary sclerosing cholangitis are a diffuse inflammation with fibrous thickening and narrowing of the biliary ducts. All patients who have stones or have been operated on for biliary disease (risk of traumatic stenosis) must be excluded in discussions of this disease. Two observations illustrating the strict definition of its characteristics are related. From attacks of angiocholitis, the disease generally progresses to a permanent jaundice. Death occurs in a state of biliary cirrhosis. Liver histopathologic changes consist of fibrous rings constricting the segmentary interlobular ducts. Physiopathologic changes of the disease are difficult to define precisely except in cases with coexistent ulcerative colitis (1/3 of cases). The diagnosis is difficult. Illustrative examples are presented: A syndrome of stenosis in a patient in whom no stones were found during the operation but the lesions were typical of the passage of stones. The prognosis was good. One patient seemed rather typical but the rapid evolution and finally a finding of carcinomatous tissue led to the diagnosis of cancer of the biliary duct. One patient had cirrhosis with extrahepatic stenosis. Histologic studies, cholangiolitis and obstructive pericholangiolitis indicated the correct diagnosis. The disease is usually fatal within four years of onset despite administration of corticoids locally and systemically.

Adult↗
PubMed1978

[Transendoscopic retrograde cholangiography in the diagnosis of primary sclerosing cholangitis. Study of 6 cases].

Primary sclerosing cholangitis (PSC) is a rare disease of unknown etiology characterized by severe chronic inflammation, fibrosis and stenosis of variable length of the extra and/or intrahepatic biliary ducts in the absence of recent operative trauma, biliary stones, cancer or infection. Diagnosis has been made only at operation. The introduction of endoscopic retrograde cholangiography (ERC) offers the possibility of preoperative diagnosis. Six cases of PSC diagnosed by ERC are presented. Characteristic roentgenologic findings include strictures of variable length of extra and intrahepatic biliary ducts, beaded appearance and decreased arborization of intrahepatic biliary tree. The roentgenological anatomy of biliary tree at ERC influence the subsequent therapy, i.e. surgical therapy is indicated if a drainage can be performed above the site of the stenosis, while medical therapy (steroids and/or immunosuppressive drugs) is the choice when intrahepatic biliary tree is involved.

Adult↗
PubMed2026

Current Management of Primary Sclerosing Cholangitis (PSC) ~A Proposal for Early-stage PSC~.

Primary sclerosing cholangitis (PSC) is a chronic, progressive cholangiopathy characterized by inflammation and fibrosis of intrahepatic and/or extrahepatic bile ducts. Its pathogenesis remains incompletely understood, and liver transplantation is currently the only curative treatment available. The diagnosis remains challenging, and no disease-specific biomarkers have been established. Recently, anti-integrin αvβ6 antibodies have emerged as promising serological biomarkers with high specificity for PSC. Advances in imaging modalities, including magnetic resonance cholangiopancreatography and peroral cholangioscopy, have improved diagnostic accuracy for PSC. Although various therapeutic approaches have been investigated, no treatment has been shown to improve the long-term outcomes. Microbiota-targeted therapies represent a promising emerging strategy. The clinical course of PSC, particularly in its early stages, is poorly defined. We propose a definition of early stage PSC consisting of two subtypes: small-duct PSC without liver fibrosis and large-duct PSC without cholestatic enzyme elevation or biliary strictures. Early intervention at this stage may improve the prognosis, thus highlighting the need for further validation.

Primary sclerosing cholangitis↗
PubMed1977

[Surgical problems of the primary sclerosing cholangitis (author's transl)].

The characteristics of the primary sclerosing cholangitis are a diffuse inflammation with fibrous thickening and narrowing of the biliar ducts. Two observations replying to strict characteristics are related. From attacks of angiocholitis, the disease generally progresses till a parmanent icterus and death occurs in a state of biliar cirrhosis. In the liver, the micro pathology consists in fibrous rings constricting the segmentary interlobular ducts. The diagnostic is mainly difficult and the prognostic is fatal in average time of 4 years of evolution althought we used corticoids that were administrated by local method as well as in the classical general way.

Adult↗
PubMed1979

The role of endoscopic retrograde cholangiography in the diagnosis and management of patients with primary sclerosing cholangitis.

The clinical and radiological findings in four patients with primary sclerosing cholangitis one of whom had coexistent cholangiocarcinoma, are reported. The need for surgical exploration to make the diagnosis was averted by the use of endoscopic retrograde cholangiography in one patient who was managed initially with medical treatment alone. Endoscopic cholangiography may be used to monitor the progress of the sclerosing lesions; but failure to fill the intrahepatic ducts is associated with a poor prognosis due either to the severity of the sclerosing process or the presence of coexistent cholangiocarcinoma.

Adult↗
PubMed2024

Identifying a therapeutic window of opportunity for people living with primary sclerosing cholangitis: Embryology and the overlap of inflammatory bowel disease with immune-mediated liver injury.

Primary sclerosing cholangitis (PSC) is a variably progressive, fibrosis-causing autoimmune disorder of the intrahepatic and extrahepatic bile ducts of unclear etiology. PSC is commonly (in 60%-90% of cases) associated with an inflammatory bowel disease (IBD) like PSC-IBD and less commonly with an autoimmune hepatitis (AIH) like PSC-AIH or AIH-overlap disorder. Hepatologists and Gastroenterologists often consider these combined conditions as distinctly different from the classical forms in isolation. Here, we review recent epidemiologic observations and highlight that PSC-IBD and PSC-AIH overlap appear to represent aspects of a common PSC clinico-pathological pathway and manifest in an age-of-presentation-dependent manner. Particularly from the pediatric experience, we hypothesize that all cases of PSC likely originate from a complex "Early PSC"-"IBD"-"AIH" overlap in which PSC defines the uniquely and variably associated "AIH" and "IBD" components along an individualized lifetime continuum. We speculate that a distinctly unique, "diverticular autoimmunity" against the embryonic cecal- and hepatic diverticulum-derived tissues may be the origin of this combined syndrome, where "AIH" and "IBD" variably commence then variably fade while PSC progresses with age. Our hypothesis provides an explanation for the age-dependent variation in the presentation and progression of PSC. This is critical for the optimal targeting of studies into PSC etiopathogenesis and emphasizes the concept of a "developmental window of opportunity for therapeutic mitigation" in what is currently recognized as an irreversible disease process. The discovery of such a window would be critically important for the targeting of interventions, both the administration of current therapies and therapeutic trial planning.

Humans↗
PubMed1979

Primary sclerosing cholangitis associated with fibrosis of the submandibular glands and the pancreas.

A new syndrome of primary sclerosing cholangitis associated with fibrosis of the submandibular glands and the pancreas is described in a 43-year-old male. The sclerosing cholangitis was diagnosed at laparotomy because of cholestasis and the fibrosis of the submandibular glands and pancreas confirmed at microscopical investigation of biopsy specimens. The cholangitis responded well to treatment with a low dose of prednisolone (7.5--10 mg) and an endoscopic retrograde cholangiopancreaticographic examination 10 months after the operation revealed normal bile ducts.

Adult↗
PubMed1979

[Two cases of primary sclerosing cholangitis (author's transl)].

Two patients with symptoms of progressive obstructive jaundice, a history of vague pains in the right upper quadrant and laboratory evidence of biliary obstruction underwent laparotomy. A stone-free, but extremely thick-walled gallbladder was found in both patients. Intraoperative cholangiography showed diffusely-stenosed extrahepatic bile ducts suggestive of chronic inflammatory changes in the biliary system. The correct diagnosis was made only on histological examination, which revealed primary sclerosing cholangitis with secondary cholestatic changes of the liver. Postoperative treatment included long-time corticosteroid therapy. Both patients have remained jaundice-free for periods of one and two years, respectively, to date, but the eventual prognosis is poor. The diagnosis, which can be made only surgically, therapy and prognosis are discussed.

Adult↗
PubMedDate not supplied

[Primary sclerosing cholangitis].

A case of intermittent cholostatic icterus has been observed at the Civil Hospital of Castellamonte. Clinical and laboratory examinations did not permit precise diagnosis, hence the decision to carry out exploratory surgery. The finding was hepato-choledochic fibrosis with severe stenosis of the lumen and patency of Vater's tubercle, with no calculosis or previous bile way operations. Transcholedochic and transpapillary biliary drainage was therefore performed with a T tube. The patient is in good condition about two years after the operation and cholangiocarcinoma can be excluded. It is therefore considered that the case can be classified as a "primary sclerosing cholangitis".

Ampulla of Vater↗
PubMed1977

Sclerosing cholangitis and primary biliary cirrhosis--a disease spectrum?

Sclerosing diseases of the biliary system encompass a spectrum ranging from primary sclerosing cholangitis (usually of the extrahepatic biliary tree) to primary biliary cirrhosis of the intrahepatic bile canaliculi. In a study of 35 patients with primary intra- and extrahepatic biliary sclerosis, age of onset, sex distribution, symptomatology, associated diseases, radiographic abnormalities and chemical profile were considered. The difficulty of differentiating sclerosing cholangitis and biliary cirrhosis from other causes of obstructive jaundice preoperatively was stressed, in addition to points of differential clinical and laboratory findings. The etiology of these entities as well as the possibility that they represent variant clinical manifestations of the same disease process were also considered. Mechanical and pharmacological treatment alternatives that were attempted included drainage procedures, the easiest and most widely used of which was the T-tube. However, this could prove to be a source of infection and should therefore be removed early, inasmuch as cholangitis represents a major cause of morbidity. Steroids have been used with varying effectiveness; subjective improvement was generally attained, although objective improvement has been difficult to document. When choleuretics and cholestyramine were administered, we noted significant palliation. Antibiotics were reserved for treatment of cholangitis. Until the underlying etiology of this rare malignant sclerosing process is found, only symptomatic treatment can be offered.

Adult↗