This is a review of the characteristic findings of inflammatory bowel disease(IBD)associated with primary sclerosing cholangitis(PSC)and their usefulness in the diagnosis of sclerosing cholangitis.PSC is a chronic inf...This is a review of the characteristic findings of inflammatory bowel disease(IBD)associated with primary sclerosing cholangitis(PSC)and their usefulness in the diagnosis of sclerosing cholangitis.PSC is a chronic inflammatory disease characterized by idiopathic fibrous obstruction and is frequently associated with IBD.IBDassociated with PSC(PSC-IBD)shows an increased incidence of pancolitis,mild symptoms,and colorectal malignancy.Although an increased incidence of pancolitis is a characteristic finding,some cases are endoscopically diagnosed as right-sided ulcerative colitis.Pathological studies have revealed that inflammation occurs more frequently in the right colon than the left colon.The frequency of rectal sparing and backwash ileitis should be investigated in a future study based on the same definition.The cholangiographic findings of immunoglobulin G4-related sclerosing cholangitis(IgG4-SC)are similar to those of PSC.The rare association between IBD and IgG4-SC and the unique characteristics of PSC-IBD are useful findings for distinguishing PSC from IgG4-SC.展开更多
This article relates to the discussion of a recent study published by Wohl et al.Primary sclerosing cholangitis(PSC)is a chronic inflammatory liver disease that affects the intra-and extrahepatic bile ducts and is str...This article relates to the discussion of a recent study published by Wohl et al.Primary sclerosing cholangitis(PSC)is a chronic inflammatory liver disease that affects the intra-and extrahepatic bile ducts and is strongly associated with ulcerative colitis(UC).Endoscopic evaluation of UC is feasible and reliable in routine clinical practice,and the Mayo endoscopic subscore(MES)is one of the most commonly used endoscopic evaluation measures for UC.Patients with PSCUC are at higher risk of developing cancer and biliary tract cancer.Endoscopic scoring alone appears unreliable,and histopathological evaluation is essential to accurately assess and make effective therapeutic decisions for PSC-UC.Therefore,we aimed to discuss the accuracy of MES in patients with UC and PSC-UC and to explore the consistency between MES and the Nancy histological index.展开更多
The concurrence of primary sclerosing cholangitis(PSC)and inflammatory bowel disease(IBD)presents a therapeutic challenge,often necessitating liver transplantation in severe cases.Paeoniflorin(PAE),known for its immun...The concurrence of primary sclerosing cholangitis(PSC)and inflammatory bowel disease(IBD)presents a therapeutic challenge,often necessitating liver transplantation in severe cases.Paeoniflorin(PAE),known for its immunomodulatory and anti-inflammatory properties but with very high-water solubility and low permeability,is formulated into a paeoniflorin/phospholipid complex microemulsion(PAE-ME)to enhance its delivery in this study.It demonstrated the PAE-ME's macrophage-regulating ability to repolarize the pro-inflammatory M1 subtype to the anti-inflammatory M2 type and reduce inflammatory cytokine release.In a PSC-IBD mouse model,PAE-ME alleviated the symptoms and regulated bile acid balance.Given the close connection and crosstalk between the liver and intestine,PAE-ME yielded a synergistic therapeutic effect on both the liver and intestinal lesions.These findings suggest a promising translational approach for complex comorbidities by acting on the liver-gut axis.展开更多
BACKGROUND Primary sclerosing cholangitis(PSC)is a long-term liver condition defined by the inflammation and scarring of the bile ducts,resulting in complications such as liver cirrhosis,portal hypertension,and cholan...BACKGROUND Primary sclerosing cholangitis(PSC)is a long-term liver condition defined by the inflammation and scarring of the bile ducts,resulting in complications such as liver cirrhosis,portal hypertension,and cholangiocarcinoma.Although PSC predominantly affects adults,the incidence in pediatric patients is rising.For individuals in the advanced stages of liver disease,liver transplantation(LT)is the sole curative treatment option.However,the recurrence of PSC in the transplanted liver,known as recurrent PSC(rPSC),remains a significant concern.AIM To identify the potential risk factors for the recurrence of PSC in pediatric patients after undergoing LT.METHODS A literature search was carried out across databases,including PubMed,Embase,Cochrane Library,and Scopus,covering studies published from 1990 through 2024.The Newcastle-Ottawa scale was utilized to assess the quality of the selected studies.Statistical analyses were conducted using RevMan 5.3 software,where the risk of recurrence was quantified using hazard ratios(HR)with 95%CI.RESULTS A total of nine reports with 2524 pediatric patients with PSC were included in this analysis.The findings revealed several important risk factors connected to the rPSC in pediatric patients who had received a liver transplant,including concurrent inflammatory bowel disease(IBD),elevated liver enzyme levels,and the presence of PSCautoimmune hepatitis(AIH)overlap syndrome(all P<0.05).No statistically significant association was found between acute allograft rejection,Epstein-Barr virus infection,and the risk of rPSC recurrence in the pediatric liver transplant recipients.CONCLUSION The present systematic review and meta-analysis have identified various risk factors associated with the recurrence of PSC in pediatric patients who underwent LT,including IBD,elevated liver enzyme levels,and PSC-AIH overlap syndrome.展开更多
MM： Persistent cholestasis is a rare complication of severe trauma or infections. Little is known about the possible pathomechanisms and the clinical course.METHODS： Secondary sclerosing cholangitis was diagnosed in...MM： Persistent cholestasis is a rare complication of severe trauma or infections. Little is known about the possible pathomechanisms and the clinical course.METHODS： Secondary sclerosing cholangitis was diagnosed in five patients with persistent jaundice after severe trauma （one burn injury, three accidents, one power current injury）. Medical charts were retrospectively reviewed with regard to possible trigger mechanisms for cholestasis, and the clinical course was recorded.RESULTS： Diagnosis of secondary sclerosing cholangitis was based in all patients on the primary sclerosing cholangitis （PSC）-Iike destruction of the intrahepatic bile ducts at cholangiography after exclusion of PSC. In four patients, arterial hypotension with subsequent ischemia may have caused the bile duct damage, whereas in the case of power current injury direct thermal damage was assumed to be the trigger mechanism. The course of secondary liver fibrosis was rapidly progressive and proceeded to liver cirrhosis in all four patients with a follow-up 〉2 years. Therapeutic possibilities were limited.CONCLUSION： Posttraumatic sderosing cholangitis is a rare but rapidly progressive disease, probably caused by ischemia of the intrahepatic bile ducts via the peribiliary capillary plexus due to arterial hypotension.Gastroenterologists should be aware of this disease in patients with persistent cholestasis after severe trauma.展开更多
AIM:To clarify the characteristic features of biliary le-sions in patients with autoimmune pancreatitis(AIP) and compare them with those of primary sclerosing cholangitis(PSC) .METHODS:The clinicopathological characte...AIM:To clarify the characteristic features of biliary le-sions in patients with autoimmune pancreatitis(AIP) and compare them with those of primary sclerosing cholangitis(PSC) .METHODS:The clinicopathological characteristics of 34 patients with sclerosing cholangitis(SC) associated with AIP were compared with those of 4 patients with PSC.RESULTS:SC with AIP occurred predominantly in el-derly men.Obstructive jaundice was the most frequent initial symptom in SC with AIP.Only SC patients with AIP had elevated serum IgG4 levels,and sclerosing diseases were more frequent in these patients.SC pa-tients with AIP responded well to steroid therapy.Seg-mental stenosis of the lower bile duct was observed only in SC patients with AIP,but a beaded and pruned-tree appearance was detected only in PSC patients.Dense infi ltration of IgG4-positive plasma cells was de-tected in the bile duct wall and the periportal area,as well as in the pancreas,of SC patients with AIP.CONCLUSION:SC with AIP is distinctly different from PSC.The two diseases can be discriminated based on cholangiopancreatographic findings and serum IgG4 levels.展开更多
To our knowledge,patients with immunoglobulin G4-related sclerosing cholangitis(IgG4-SC)associated with autoimmune hemolytic anemia(AIHA)have not been reported previously.Many patients with IgG4-SC have autoimmune pan...To our knowledge,patients with immunoglobulin G4-related sclerosing cholangitis(IgG4-SC)associated with autoimmune hemolytic anemia(AIHA)have not been reported previously.Many patients with IgG4-SC have autoimmune pancreatitis(AIP)and respond to steroid treatment.However,isolated cases of IgG4-SC are difficult to diagnose.We describe our experience with a patient who had IgG4-SC without AIP in whom the presence of AIHA led to diagnosis.The patient was a73-year-old man who was being treated for dementia.Liver dysfunction was diagnosed on blood tests at another hospital.Imaging studies suggested the presence of carcinoma of the hepatic hilus and primary sclerosing cholangitis,but a rapidly progressing anemia developed simultaneously.After the diagnosis of AIHA,steroid treatment was begun,and the biliary stricture improved.IgG4-SC without AIP was thus diagnosed.展开更多
Primary sclerosing cholangitis(PSC) is a chronic progressive liver disease. Subtypes of PSC have been described, most recently PSC with elevated serum and/or tissue IgG4 subclass. We aim to summarise the clinical phen...Primary sclerosing cholangitis(PSC) is a chronic progressive liver disease. Subtypes of PSC have been described, most recently PSC with elevated serum and/or tissue IgG4 subclass. We aim to summarise the clinical phenotype,disease associations, differential diagnosis, response to therapy and pathogenic mechanisms underlying PSC-high IgG4 subtype. We reviewed Pub Med,MEDLINE and Embase with the search terms "primary sclerosing cholangitis","IgG4", and "IgG4-related sclerosing cholangitis(IgG4-SC)". Elevated serum IgG4 are found in up-to one-quarter, and abundant IgG4-plasma cell infiltrates in the liver and bile ducts are found in up-to one-fifth of PSC patients. This group have a distinct clinical phenotype, with some studies reporting a more aggressive course of liver and associated inflammatory bowel disease, compared to PSCnormal IgG4 and the disease mimic IgG4-SC. Distinguishing PSC-high IgG4 from IgG4-SC remains challenging, requiring careful assessment of clinical features,organ involvement and tissue morphology. Calculation of serum IgG4:IgG1 ratios and use of a novel IgG4:IgG RNA ratio have been reported to have excellent specificity to distinguish IgG4-SC and PSC-high IgG4 but require validation in larger cohorts. A role for corticosteroid therapy in PSC-high IgG4 remains unanswered, with concerns of increased toxicity and lack of outcome data. The immunological drivers underlying prominent IgG4 antibodies in PSC are incompletely defined. An association with PSC-high IgG4 and HLA class-II haplotypes(B*07, DRB1*15), T-helper2 and T-regulatory cytokines(IL4, IL10,IL13) and chemokines(CCL1, CCR8) have been described. PSC-high IgG4 have a distinct clinical phenotype and need careful discrimination from IgG4-SC,although response to immunosuppressive treatments and long-term outcome remains unresolved. The presence of IgG4 likely represents chronic activation to persistent antigenic exposure in genetically predisposed individuals.展开更多
Eosinophilic cholangiopathy is a rare condition characterized by eosinophilic infiltration of the biliary tract and causes sclerosing cholangitis. We report a patient with secondary sclerosing cholangitis with eosinop...Eosinophilic cholangiopathy is a rare condition characterized by eosinophilic infiltration of the biliary tract and causes sclerosing cholangitis. We report a patient with secondary sclerosing cholangitis with eosinophilic cholecystitis. A 46-year-old Japanese man was admitted to our hospital with jaundice. Computed tomography revealed dilatation of both the intrahepatic and extrahepatic bile ducts, diffuse thickening of the wall of the extrahepatic bile duct, and thickening of the gallbladder wall. Under the diagnosis of lower bile duct carcinoma, he underwent pyloruspreserving pancreatoduodenectomy and liver biopsy. On histopathological examination, conspicuous fibrosis was seen in the lower bile duct wall. In the gallbladder wall, marked eosinophilic infiltration was seen. Liver biopsy revealed mild portal fibrosis. He was diagnosed as definite eosinophilic cholecystitis with sclerosing cholangitis with unknown etiology. The possible etiology of sderosing cholangitis was consequent fibrosis from previous eosinophilic infiltration in the bile duct. The clinicopathological findings of our case and a literature review indicated that eosinophilic cholangiopathy could cause a condition mimicking primary sclerosing cholangitis （PSC）. Bile duct wall thickening in patients with eosinophilic cholangitis might be due to fibrosis of the bile duct wall. Eosinophilic cholangiopathy might be confused as PSC with eosinophilia.展开更多
BACKGROUND: Sclerosing cholangitis(SC) is a chronic cholestatic hepatobiliary disease with uncertain long-term prognosis in pediatric patients. This study aimed to evaluate longterm results in children with SC accordi...BACKGROUND: Sclerosing cholangitis(SC) is a chronic cholestatic hepatobiliary disease with uncertain long-term prognosis in pediatric patients. This study aimed to evaluate longterm results in children with SC according to the types of SC.METHODS: We retrospectively followed up 25 children with SC over a period of 4-17 years(median 12). The diagnosis of SC was based on biochemical, histological and cholangiographic findings. Patients fulfilling diagnostic criteria for probable or definite autoimmune hepatitis at the time of diagnosis were defined as having autoimmune sclerosing cholangitis(ASC); other patients were included in a group of primary sclerosing cholangitis(PSC). The incidence of the following complications was studied: obstructive cholangitis, portal hypertension, advanced liver disease and death associated with the primary disease.RESULTS: Fourteen(56%) patients had PSC and 11(44%) had ASC. Patients with ASC were significantly younger at the time of diagnosis(12.3 vs 15.4 years, P=0.032) and had higher Ig G levels(22.7 vs 17.2 g/L, P=0.003). The mentioned complications occurred in 4(16%) patients with SC, exclusively in the PSC group: one patient died from colorectal cancer, one patient underwent liver transplantation and two patients, in whom severe bile duct stenosis was present at diagnosis, were endoscopically treated for acute cholangitis. Furthermore, twoother children with ASC and 2 children with PSC had elevated aminotransferase levels. The 10-year overall survival was 95.8% in all patients, 100% in patients without complicated liver disease, and 75.0% in patients with complications.CONCLUSION: In children, ASC is a frequent type of SC, whose prognosis may be better than that in patients with PSC.展开更多
The natural history,associations with inflammatory bowel disease(IBD),and long-term outcomes of large duct primary sclerosing cholangitis(ldPSC)have been well documented.Small duct primary sclerosing cholangitis(sdPSC...The natural history,associations with inflammatory bowel disease(IBD),and long-term outcomes of large duct primary sclerosing cholangitis(ldPSC)have been well documented.Small duct primary sclerosing cholangitis(sdPSC)is a much less common and relatively more benign variant.The natural history of sdPSC has been difficult to characterize given the limited number of studies in the literature especially with regards to the subset of patients who progress to large duct involvement.It has been unclear whether sdPSC represented a subset of ldPSC,an earlier staging of ldPSC,or a completely separate and distinct entity of its own.Strong associations between sdPSC and IBD have been established with suspicion that concurrent sdPSC-IBD may be a key prognostic factor in determining which patients are at risk of progression to ldPSC.Little is known regarding the discrete circumstances that predisposes some patients with sdPSC to progress to ldPSC.It has been suspected that progression to large biliary duct involvement subjects this subset of patients to potentially developing lifethreatening complications.Here the authors conducted a thorough review of the published sdPSC literature using Pubmed searches and cross-referencing to compile all accessible studies regarding cohorts of sdPSC patients in order better characterize the subset of sdPSC patients who progress to ldPSC and the associated outcomes.展开更多
In our case, we present a case of an 80-year-old male who was referred to the gastroenterologist for evaluation of a suspicious mass. CT imaging at the time had shown intrahepatic and extrahepatic biliary dilations, a...In our case, we present a case of an 80-year-old male who was referred to the gastroenterologist for evaluation of a suspicious mass. CT imaging at the time had shown intrahepatic and extrahepatic biliary dilations, and the patient was thought to have a pancreatic or a common bile duct mass. A mass biopsy showed no malignancy, and further evaluation was warranted. The patient was found to have elevated IgG4 levels and was diagnosed with IgG4-sclerosing cholangitis (IgG4-SC). IgG4 has been found to create a wide array of pathologies, including autoimmune pancreatitis, dacryoadenitis, and sialadenitis. These pathologies have been grouped under an IgG4-Related Disease (IgG4-RD) category. In some cases, this IgG4-RD can present as a subclass of primary sclerosing cholangitis due to immune depositions and swelling of the CBD. Due to the strictures caused by the sclerosing cholangitis, intrahepatic and extrahepatic dilations might be found on endoscopic ultrasound (EUS). It is imperative to differentiate this from a malignant mass as the early recognition and treatment of IgG4-SC can lead to complete resolution. In this case report, we present a case of a patient who was found to have IgG4-SC and responded well to steroid treatment.展开更多
Chronic hepatobiliary inflammatory diseases are not widely acknowledged as underlying disorders of systemic AA amyloidosis,except epidemic schistosomiasis.Among them,primary sclerosing cholangitis (PSC) might initiate...Chronic hepatobiliary inflammatory diseases are not widely acknowledged as underlying disorders of systemic AA amyloidosis,except epidemic schistosomiasis.Among them,primary sclerosing cholangitis (PSC) might initiate amyloid A protein deposition in diverse tissues,giving rise to systemic amyloidosis,due to a progressive and unresolved inflammatory process,and its possible association with inflammatory bowel diseases.Nevertheless,only one such case has been reported in the literature to date.We report a 69-year-old Japanese woman with cirrhosis who was diagnosed with PSC complicated with systemic AA amyloidosis,without any evidence of other inflammatory disorders.As a result of cholestasis in conjunction with biliary strictures and increased serum IgG4,the presence of IgG4 + plasma cells was examined systemically,resulting in unexpected documentation of Congo-red-positive amyloid deposits,but not IgG4 + plasma cells,in the liver,stomach and salivary glands.Elevated serum IgG4 is the hallmark of IgG4-related disease,including IgG4-associated cholangitis,but it has also been demonstrated in certain patients with PSC.Amyloid A deposits in multiple organs associated with an indolent clinical course that progresses over many years might have a diagnostic value in discriminating PSC from IgG4-associated cholangitis.展开更多
IgG4-related sclerosing cholangitis(IgG4-SC)is often associated with autoimmune pancreatitis.However,the diffuse cholangiographic abnormalities observed in IgG4-SC may resemble those observed in primary sclerosing cho...IgG4-related sclerosing cholangitis(IgG4-SC)is often associated with autoimmune pancreatitis.However,the diffuse cholangiographic abnormalities observed in IgG4-SC may resemble those observed in primary sclerosing cholangitis(PSC),and the presence of segmental stenosis suggests cholangiocarcinoma(CC).IgG4-SC responds well to steroid therapy,whereas PSC is only effectively treated with liver transplantation and CC requires surgical intervention.Since IgG4-SC was first described,it has become a third distinct clinical entity of sclerosing cholangitis.The aim of this review was to introduce the diagnostic methods for IgG4-SC.IgG4-SC should be carefully diagnosed based on a combination of characteristic clinical,serological,morphological,and histopathological features after cholangiographic classification and targeting of a disease for differential diagnosis.When intrapancreatic stenosis is detected,pancreatic cancer or CC should be ruled out.If multiple intrahepatic stenoses are evident,PSC should be distinguished on the basis of cholangiographic findings and liver biopsy with IgG4 immunostaining.Associated inflammatory bowel disease is suggestive of PSC.If stenosis is demonstrated in the hepatic hilar region,CC should be discriminated by ultrasonography,intraductal ultrasonography,bile duct biopsy,and a higher cutoff serum IgG4 level of 182 mg/dL.展开更多
AIM:To review the current literature for the specificclinical characteristics of inflammatory bowel disease(IBD)associated with primary sclerosing cholangitis(PSC).METHODS:A systematical review for clinical characteri...AIM:To review the current literature for the specificclinical characteristics of inflammatory bowel disease(IBD)associated with primary sclerosing cholangitis(PSC).METHODS:A systematical review for clinical characteristics of IBD in PSC was performed by conducting a broad search for"primary sclerosing cholangitis"in Pubmed."Clinical characteristics"were specified into five predefined subthemes:epidemiology of IBD in PSC,characteristics of IBD in PSC(i.e.,location,disease behavior),risk of colorectal cancer development,IBD recurrence and de novo disease after liver transplantation for PSC,and safety and complications after proctocolectomy with ileal pouchanal anastomosis.Papers were selected for inclusion based on their relevance to the subthemes,and were reviewed by two independent reviewers.Only full papers relevant to PSC-IBD were included.Additionally the references of recent reviews for PSC(<5 years old)were scrutinized for relevant articles.RESULTS:Initial literature search for PSC yielded 4704results.After careful review 65 papers,comprising a total of 11406 PSC-IBD patients,were selected and divided according to subtheme.Four manuscripts overlapped and were included in two subthemes.Prevalence of IBD in PSC shows a large variance,ranging from 46.5%to 98.7%with ulcerative colitis(UC)being the most common type(>75%).The highest IBD rates in PSC are found in papers reviewing both endoscopic and histological data for IBD diagnosis.Although IBD in PSC is found to be a quiescent disease,pancolitis occurs often,with rates varying from 35%to 95%.Both backwash ileitis and rectal sparing are observed infrequently.The development of dysplasia or colorectal carcinoma is increased in PSC-IBD;the cumulative 10 years risk varying between 0%and11%.Exacerbation of IBD is common after liver transplantation for PSC and de novo disease is seen in1.3%to 31.3%of PSC-IBD patients.The risk for development of pouchitis in PSC-IBD is found to besignificant,affecting 13.8%to 90%of the patients after proctocolectomy with ileo anal-pouch anastomosis.CONCLUSION:IBD in primary sclerosing cholangitis represents a distinct phenotype that differs from UC and Crohn’s disease and therefore requires specialized management.展开更多
Primary sclerosing cholangitis (PSC) is a chronic progressive inflammatory disease affecting the bile ducts, leading to f ibrosis and eventually cirrhosis in most patients. Its etiology is unknown and so far no effect...Primary sclerosing cholangitis (PSC) is a chronic progressive inflammatory disease affecting the bile ducts, leading to f ibrosis and eventually cirrhosis in most patients. Its etiology is unknown and so far no effective medical therapy is available. Liver transplantation (LTX) is the only curative treatment and at present PSC is the main indication for LTX in the Scandinavian countries. Close to half of the PSC patients experience one or more episodes of acute cellular rejection (ACR) following transplantation and approximately 1/5 of the transplanted patients develop recurrent disease in the graft. In addition, some reports indicate that ACR early after LTX for PSC can infl uence the risk for recurrent disease. For these important post-transplantation entities affecting PSC patients, we have reviewed the current literature on epidemiology, pathogenesis, treatment and the possible infl uence of rejection on the risk of recurrent disease in the allograft.展开更多
To examine and evaluate recent evidence regarding the epidemiology, pathogenesis and management of colorectal cancer(CRC) development in inflammatory bowel disease(IBD)-primary sclerosing cholangitis(PSC) patients. Us...To examine and evaluate recent evidence regarding the epidemiology, pathogenesis and management of colorectal cancer(CRC) development in inflammatory bowel disease(IBD)-primary sclerosing cholangitis(PSC) patients. Using the PubMed database, a literature search was conducted for relevant articles in English from the past 10 years. Relevant studies investigating PSC as a risk factor for CRC in IBD in the context of incidence and prevalence, pathogenesis, prevention and prognosis were included in this review. Recent evidence increasingly points to PSC as a significant risk factor in the development of CRC in patients with concomitant IBD. PSC may be an important risk factor for CRC in different populations worldwide. The mechanism for this increase in risk is still unclear. The efficacy of UDCA as a chemopreventive agent remains controversial. Liver transplantation does not halt the development of CRC, although there is not enough evidence to suggest that it is associated with increased incidence of CRC. While routine colonoscopic surveillance should be performed in patients with concurrent PSC and IBD, more high-level evidence is required to support the benefits of the procedure. While many new developments have taken place in the last decade, the pathogenesis and optimal management of CRC development in IBD-PSC patients remain unclear.展开更多
Sclerosing cholangitis(SC)is a rarely reported morbidity secondary to transcatheter arterial chemoembolization(TACE)with bleomycin-iodinated oil(BIO)for liver cavernous hemangioma(LCH).This report retrospectively eval...Sclerosing cholangitis(SC)is a rarely reported morbidity secondary to transcatheter arterial chemoembolization(TACE)with bleomycin-iodinated oil(BIO)for liver cavernous hemangioma(LCH).This report retrospectively evaluated the diagnostic and therapeutic course of a patient with LDH who presented obstructive jaundice 6 years after TACE with BIO.Preoperative imaging identified a suspected malignant biliary stricture located at the convergence of the left and right hepatic ducts.Operative exploration demonstrated a full-thickness sclerosis of the hilar bile duct with right hepatic duct stricture and right lobe atrophy.Radical hepatic hilar resection with right-side hemihepatectomy and Roux-en-Y hepaticojejunostomy was performed because hilar cancer could not be excluded on frozen biopsy.Pathological results showed chronic pyogenic inflammation of the common and right hepatic ducts with SC in the portal area.Secondary SC is a long-term complication that may occur in LCH patients after TACE with BIO and must be differentiated from hilar malignancy.Hepatic duct plasty is a definitive but technically challenging treatment modality for secondary SC.展开更多
Patients with primary sclerosing cholangitis(PSC) complicated by inflammatory bowel disease(IBD) represent a distinct subset of patients with unique characteristics,which have serious clinical implications.The aim of ...Patients with primary sclerosing cholangitis(PSC) complicated by inflammatory bowel disease(IBD) represent a distinct subset of patients with unique characteristics,which have serious clinical implications.The aim of this literature review was to shed light to the obscure clinical and molecular aspects of the two diseases combined utilizing current data available and putting issues of diagnosis and treatment into perspective.The prevalence of IBD,mainly ulcerative colitis in PSC patients is estimated to be 21%-80%,dependent on screening programs and nationality.PSC-associated colitis is likely to be extensive,characterized by rectal sparing,backwash ileitis,and generally mild symptoms.It is also more likely to progress to colorectal malignancy,making it imperative for clinicians to maintain a high level of suspicion when tackling PSC patients.There is no optimal surveillance strategy but current guidelines advocate that colonoscopy is necessary at the time of PSC diagnosis with annual endoscopic follow-up.Random biopsies have been criticized and a shift towards targeted biopsies using chromoendoscopy,laser endomicroscopy and narrow-band imaging has been noted.Techniques directed towards genetic mutations instead of histological abnormalities hold promise for easier,more accurate diagnosis of dysplastic lesions.Chemopreventive measures against colorectal cancer have been sought in these patients.Ursodeoxycholic acid seemed promising at first but subsequent studies yielded conflicting results showing anticarcinogenic effects in low doses(8-15 mg/kg per day) and carcinogenic properties in high doses(15-30 mg/kg per day).展开更多
To characterize the gut bacterial microbiota of patients with primary sclerosing cholangitis(PSC)and ulcerative colitis(UC).METHODSStool samples were collected and relevant clinical data obtained from 106 study partic...To characterize the gut bacterial microbiota of patients with primary sclerosing cholangitis(PSC)and ulcerative colitis(UC).METHODSStool samples were collected and relevant clinical data obtained from 106 study participants,43 PSC patients with(n=32)or without(n=11)concomitant inflammatory bowel disease,32 UC patients,and 31 healthy controls.The V3 and V4 regions of the 16S ribosomal RNA gene were sequenced on Illumina MiSeq platform to cover low taxonomic levels.Data were further processed in QIIME employing MaAsLin and LEfSe tools for analysis of the output data.RESULTSMicrobial profiles in both PSC and UC were characterized by low bacterial diversity and significant change in global microbial composition.Rothia,Enterococcus,Streptococcus,Veillonella,and three other genera were markedly overrepresented in PSC regardless of concomitant inflammatory bowel disease(IBD).Rothia,Veillonella and Streptococcus were tracked to the species level to identify Rothia mucilaginosa,Streptococcus infantus,S.alactolyticus,and S.equi along with Veillonella parvula and V.dispar.PSC was further characterized by decreased abundance of Adlercreutzia equolifaciens and Prevotella copri.Decrease in genus Phascolarctobacterium was linked to presence of colonic inflammation regardless of IBD phenotype.Akkermansia muciniphila,Butyricicoccus pullicaecorum and Clostridium colinum were decreased in UC along with genus Roseburia.Low levels of serum albumin were significantly correlated with enrichment of order Actinomycetales.CONCLUSIONPSC is associated with specific gut microbes independently of concomitant IBD and several bacterial taxa clearly distinguish IBD phenotypes(PSC-IBD and UC).展开更多
文摘This is a review of the characteristic findings of inflammatory bowel disease(IBD)associated with primary sclerosing cholangitis(PSC)and their usefulness in the diagnosis of sclerosing cholangitis.PSC is a chronic inflammatory disease characterized by idiopathic fibrous obstruction and is frequently associated with IBD.IBDassociated with PSC(PSC-IBD)shows an increased incidence of pancolitis,mild symptoms,and colorectal malignancy.Although an increased incidence of pancolitis is a characteristic finding,some cases are endoscopically diagnosed as right-sided ulcerative colitis.Pathological studies have revealed that inflammation occurs more frequently in the right colon than the left colon.The frequency of rectal sparing and backwash ileitis should be investigated in a future study based on the same definition.The cholangiographic findings of immunoglobulin G4-related sclerosing cholangitis(IgG4-SC)are similar to those of PSC.The rare association between IBD and IgG4-SC and the unique characteristics of PSC-IBD are useful findings for distinguishing PSC from IgG4-SC.
文摘This article relates to the discussion of a recent study published by Wohl et al.Primary sclerosing cholangitis(PSC)is a chronic inflammatory liver disease that affects the intra-and extrahepatic bile ducts and is strongly associated with ulcerative colitis(UC).Endoscopic evaluation of UC is feasible and reliable in routine clinical practice,and the Mayo endoscopic subscore(MES)is one of the most commonly used endoscopic evaluation measures for UC.Patients with PSCUC are at higher risk of developing cancer and biliary tract cancer.Endoscopic scoring alone appears unreliable,and histopathological evaluation is essential to accurately assess and make effective therapeutic decisions for PSC-UC.Therefore,we aimed to discuss the accuracy of MES in patients with UC and PSC-UC and to explore the consistency between MES and the Nancy histological index.
基金National Key Research and Development Program of China(No.2022YFE0203600,China)National Natural Science Foundation of China(Nos.82341232,81925035)+3 种基金Department of Science and Technology of Guangdong Province(No.2021B0909050003)Chinese Academy of Sciences President's International Fellowship Initiative(No.2024VBB0004)the Scientific Innovation Group Project in Zhongshan(No.CXTD2022011)supported by grants from the Program of Shanghai Committee of Science and Technology,China(No.22S21902900)。
文摘The concurrence of primary sclerosing cholangitis(PSC)and inflammatory bowel disease(IBD)presents a therapeutic challenge,often necessitating liver transplantation in severe cases.Paeoniflorin(PAE),known for its immunomodulatory and anti-inflammatory properties but with very high-water solubility and low permeability,is formulated into a paeoniflorin/phospholipid complex microemulsion(PAE-ME)to enhance its delivery in this study.It demonstrated the PAE-ME's macrophage-regulating ability to repolarize the pro-inflammatory M1 subtype to the anti-inflammatory M2 type and reduce inflammatory cytokine release.In a PSC-IBD mouse model,PAE-ME alleviated the symptoms and regulated bile acid balance.Given the close connection and crosstalk between the liver and intestine,PAE-ME yielded a synergistic therapeutic effect on both the liver and intestinal lesions.These findings suggest a promising translational approach for complex comorbidities by acting on the liver-gut axis.
文摘BACKGROUND Primary sclerosing cholangitis(PSC)is a long-term liver condition defined by the inflammation and scarring of the bile ducts,resulting in complications such as liver cirrhosis,portal hypertension,and cholangiocarcinoma.Although PSC predominantly affects adults,the incidence in pediatric patients is rising.For individuals in the advanced stages of liver disease,liver transplantation(LT)is the sole curative treatment option.However,the recurrence of PSC in the transplanted liver,known as recurrent PSC(rPSC),remains a significant concern.AIM To identify the potential risk factors for the recurrence of PSC in pediatric patients after undergoing LT.METHODS A literature search was carried out across databases,including PubMed,Embase,Cochrane Library,and Scopus,covering studies published from 1990 through 2024.The Newcastle-Ottawa scale was utilized to assess the quality of the selected studies.Statistical analyses were conducted using RevMan 5.3 software,where the risk of recurrence was quantified using hazard ratios(HR)with 95%CI.RESULTS A total of nine reports with 2524 pediatric patients with PSC were included in this analysis.The findings revealed several important risk factors connected to the rPSC in pediatric patients who had received a liver transplant,including concurrent inflammatory bowel disease(IBD),elevated liver enzyme levels,and the presence of PSCautoimmune hepatitis(AIH)overlap syndrome(all P<0.05).No statistically significant association was found between acute allograft rejection,Epstein-Barr virus infection,and the risk of rPSC recurrence in the pediatric liver transplant recipients.CONCLUSION The present systematic review and meta-analysis have identified various risk factors associated with the recurrence of PSC in pediatric patients who underwent LT,including IBD,elevated liver enzyme levels,and PSC-AIH overlap syndrome.
文摘MM： Persistent cholestasis is a rare complication of severe trauma or infections. Little is known about the possible pathomechanisms and the clinical course.METHODS： Secondary sclerosing cholangitis was diagnosed in five patients with persistent jaundice after severe trauma （one burn injury, three accidents, one power current injury）. Medical charts were retrospectively reviewed with regard to possible trigger mechanisms for cholestasis, and the clinical course was recorded.RESULTS： Diagnosis of secondary sclerosing cholangitis was based in all patients on the primary sclerosing cholangitis （PSC）-Iike destruction of the intrahepatic bile ducts at cholangiography after exclusion of PSC. In four patients, arterial hypotension with subsequent ischemia may have caused the bile duct damage, whereas in the case of power current injury direct thermal damage was assumed to be the trigger mechanism. The course of secondary liver fibrosis was rapidly progressive and proceeded to liver cirrhosis in all four patients with a follow-up 〉2 years. Therapeutic possibilities were limited.CONCLUSION： Posttraumatic sderosing cholangitis is a rare but rapidly progressive disease, probably caused by ischemia of the intrahepatic bile ducts via the peribiliary capillary plexus due to arterial hypotension.Gastroenterologists should be aware of this disease in patients with persistent cholestasis after severe trauma.
文摘AIM:To clarify the characteristic features of biliary le-sions in patients with autoimmune pancreatitis(AIP) and compare them with those of primary sclerosing cholangitis(PSC) .METHODS:The clinicopathological characteristics of 34 patients with sclerosing cholangitis(SC) associated with AIP were compared with those of 4 patients with PSC.RESULTS:SC with AIP occurred predominantly in el-derly men.Obstructive jaundice was the most frequent initial symptom in SC with AIP.Only SC patients with AIP had elevated serum IgG4 levels,and sclerosing diseases were more frequent in these patients.SC pa-tients with AIP responded well to steroid therapy.Seg-mental stenosis of the lower bile duct was observed only in SC patients with AIP,but a beaded and pruned-tree appearance was detected only in PSC patients.Dense infi ltration of IgG4-positive plasma cells was de-tected in the bile duct wall and the periportal area,as well as in the pancreas,of SC patients with AIP.CONCLUSION:SC with AIP is distinctly different from PSC.The two diseases can be discriminated based on cholangiopancreatographic findings and serum IgG4 levels.
文摘To our knowledge,patients with immunoglobulin G4-related sclerosing cholangitis(IgG4-SC)associated with autoimmune hemolytic anemia(AIHA)have not been reported previously.Many patients with IgG4-SC have autoimmune pancreatitis(AIP)and respond to steroid treatment.However,isolated cases of IgG4-SC are difficult to diagnose.We describe our experience with a patient who had IgG4-SC without AIP in whom the presence of AIHA led to diagnosis.The patient was a73-year-old man who was being treated for dementia.Liver dysfunction was diagnosed on blood tests at another hospital.Imaging studies suggested the presence of carcinoma of the hepatic hilus and primary sclerosing cholangitis,but a rapidly progressing anemia developed simultaneously.After the diagnosis of AIHA,steroid treatment was begun,and the biliary stricture improved.IgG4-SC without AIP was thus diagnosed.
基金Supported by the National Institute of Health Research Biomedical Research Centre,based at Oxford University Hospitals TrustOxfordshire Health Service Research Committee as part of Oxford Hospitals Charity,Oxford。
文摘Primary sclerosing cholangitis(PSC) is a chronic progressive liver disease. Subtypes of PSC have been described, most recently PSC with elevated serum and/or tissue IgG4 subclass. We aim to summarise the clinical phenotype,disease associations, differential diagnosis, response to therapy and pathogenic mechanisms underlying PSC-high IgG4 subtype. We reviewed Pub Med,MEDLINE and Embase with the search terms "primary sclerosing cholangitis","IgG4", and "IgG4-related sclerosing cholangitis(IgG4-SC)". Elevated serum IgG4 are found in up-to one-quarter, and abundant IgG4-plasma cell infiltrates in the liver and bile ducts are found in up-to one-fifth of PSC patients. This group have a distinct clinical phenotype, with some studies reporting a more aggressive course of liver and associated inflammatory bowel disease, compared to PSCnormal IgG4 and the disease mimic IgG4-SC. Distinguishing PSC-high IgG4 from IgG4-SC remains challenging, requiring careful assessment of clinical features,organ involvement and tissue morphology. Calculation of serum IgG4:IgG1 ratios and use of a novel IgG4:IgG RNA ratio have been reported to have excellent specificity to distinguish IgG4-SC and PSC-high IgG4 but require validation in larger cohorts. A role for corticosteroid therapy in PSC-high IgG4 remains unanswered, with concerns of increased toxicity and lack of outcome data. The immunological drivers underlying prominent IgG4 antibodies in PSC are incompletely defined. An association with PSC-high IgG4 and HLA class-II haplotypes(B*07, DRB1*15), T-helper2 and T-regulatory cytokines(IL4, IL10,IL13) and chemokines(CCL1, CCR8) have been described. PSC-high IgG4 have a distinct clinical phenotype and need careful discrimination from IgG4-SC,although response to immunosuppressive treatments and long-term outcome remains unresolved. The presence of IgG4 likely represents chronic activation to persistent antigenic exposure in genetically predisposed individuals.
文摘Eosinophilic cholangiopathy is a rare condition characterized by eosinophilic infiltration of the biliary tract and causes sclerosing cholangitis. We report a patient with secondary sclerosing cholangitis with eosinophilic cholecystitis. A 46-year-old Japanese man was admitted to our hospital with jaundice. Computed tomography revealed dilatation of both the intrahepatic and extrahepatic bile ducts, diffuse thickening of the wall of the extrahepatic bile duct, and thickening of the gallbladder wall. Under the diagnosis of lower bile duct carcinoma, he underwent pyloruspreserving pancreatoduodenectomy and liver biopsy. On histopathological examination, conspicuous fibrosis was seen in the lower bile duct wall. In the gallbladder wall, marked eosinophilic infiltration was seen. Liver biopsy revealed mild portal fibrosis. He was diagnosed as definite eosinophilic cholecystitis with sclerosing cholangitis with unknown etiology. The possible etiology of sderosing cholangitis was consequent fibrosis from previous eosinophilic infiltration in the bile duct. The clinicopathological findings of our case and a literature review indicated that eosinophilic cholangiopathy could cause a condition mimicking primary sclerosing cholangitis （PSC）. Bile duct wall thickening in patients with eosinophilic cholangitis might be due to fibrosis of the bile duct wall. Eosinophilic cholangiopathy might be confused as PSC with eosinophilia.
基金supported by a grant from the Czech Ministry of Education,Youth and Sports(NPU LO 1304)
文摘BACKGROUND: Sclerosing cholangitis(SC) is a chronic cholestatic hepatobiliary disease with uncertain long-term prognosis in pediatric patients. This study aimed to evaluate longterm results in children with SC according to the types of SC.METHODS: We retrospectively followed up 25 children with SC over a period of 4-17 years(median 12). The diagnosis of SC was based on biochemical, histological and cholangiographic findings. Patients fulfilling diagnostic criteria for probable or definite autoimmune hepatitis at the time of diagnosis were defined as having autoimmune sclerosing cholangitis(ASC); other patients were included in a group of primary sclerosing cholangitis(PSC). The incidence of the following complications was studied: obstructive cholangitis, portal hypertension, advanced liver disease and death associated with the primary disease.RESULTS: Fourteen(56%) patients had PSC and 11(44%) had ASC. Patients with ASC were significantly younger at the time of diagnosis(12.3 vs 15.4 years, P=0.032) and had higher Ig G levels(22.7 vs 17.2 g/L, P=0.003). The mentioned complications occurred in 4(16%) patients with SC, exclusively in the PSC group: one patient died from colorectal cancer, one patient underwent liver transplantation and two patients, in whom severe bile duct stenosis was present at diagnosis, were endoscopically treated for acute cholangitis. Furthermore, twoother children with ASC and 2 children with PSC had elevated aminotransferase levels. The 10-year overall survival was 95.8% in all patients, 100% in patients without complicated liver disease, and 75.0% in patients with complications.CONCLUSION: In children, ASC is a frequent type of SC, whose prognosis may be better than that in patients with PSC.
文摘The natural history,associations with inflammatory bowel disease(IBD),and long-term outcomes of large duct primary sclerosing cholangitis(ldPSC)have been well documented.Small duct primary sclerosing cholangitis(sdPSC)is a much less common and relatively more benign variant.The natural history of sdPSC has been difficult to characterize given the limited number of studies in the literature especially with regards to the subset of patients who progress to large duct involvement.It has been unclear whether sdPSC represented a subset of ldPSC,an earlier staging of ldPSC,or a completely separate and distinct entity of its own.Strong associations between sdPSC and IBD have been established with suspicion that concurrent sdPSC-IBD may be a key prognostic factor in determining which patients are at risk of progression to ldPSC.Little is known regarding the discrete circumstances that predisposes some patients with sdPSC to progress to ldPSC.It has been suspected that progression to large biliary duct involvement subjects this subset of patients to potentially developing lifethreatening complications.Here the authors conducted a thorough review of the published sdPSC literature using Pubmed searches and cross-referencing to compile all accessible studies regarding cohorts of sdPSC patients in order better characterize the subset of sdPSC patients who progress to ldPSC and the associated outcomes.
文摘In our case, we present a case of an 80-year-old male who was referred to the gastroenterologist for evaluation of a suspicious mass. CT imaging at the time had shown intrahepatic and extrahepatic biliary dilations, and the patient was thought to have a pancreatic or a common bile duct mass. A mass biopsy showed no malignancy, and further evaluation was warranted. The patient was found to have elevated IgG4 levels and was diagnosed with IgG4-sclerosing cholangitis (IgG4-SC). IgG4 has been found to create a wide array of pathologies, including autoimmune pancreatitis, dacryoadenitis, and sialadenitis. These pathologies have been grouped under an IgG4-Related Disease (IgG4-RD) category. In some cases, this IgG4-RD can present as a subclass of primary sclerosing cholangitis due to immune depositions and swelling of the CBD. Due to the strictures caused by the sclerosing cholangitis, intrahepatic and extrahepatic dilations might be found on endoscopic ultrasound (EUS). It is imperative to differentiate this from a malignant mass as the early recognition and treatment of IgG4-SC can lead to complete resolution. In this case report, we present a case of a patient who was found to have IgG4-SC and responded well to steroid treatment.
基金Supported by Grants-in-Aid for Clinical Research from National Hospital Organization,Grants-in-Aid for Scientific Research from the Ministry of Health,Labour and Welfare of Japan
文摘Chronic hepatobiliary inflammatory diseases are not widely acknowledged as underlying disorders of systemic AA amyloidosis,except epidemic schistosomiasis.Among them,primary sclerosing cholangitis (PSC) might initiate amyloid A protein deposition in diverse tissues,giving rise to systemic amyloidosis,due to a progressive and unresolved inflammatory process,and its possible association with inflammatory bowel diseases.Nevertheless,only one such case has been reported in the literature to date.We report a 69-year-old Japanese woman with cirrhosis who was diagnosed with PSC complicated with systemic AA amyloidosis,without any evidence of other inflammatory disorders.As a result of cholestasis in conjunction with biliary strictures and increased serum IgG4,the presence of IgG4 + plasma cells was examined systemically,resulting in unexpected documentation of Congo-red-positive amyloid deposits,but not IgG4 + plasma cells,in the liver,stomach and salivary glands.Elevated serum IgG4 is the hallmark of IgG4-related disease,including IgG4-associated cholangitis,but it has also been demonstrated in certain patients with PSC.Amyloid A deposits in multiple organs associated with an indolent clinical course that progresses over many years might have a diagnostic value in discriminating PSC from IgG4-associated cholangitis.
文摘IgG4-related sclerosing cholangitis(IgG4-SC)is often associated with autoimmune pancreatitis.However,the diffuse cholangiographic abnormalities observed in IgG4-SC may resemble those observed in primary sclerosing cholangitis(PSC),and the presence of segmental stenosis suggests cholangiocarcinoma(CC).IgG4-SC responds well to steroid therapy,whereas PSC is only effectively treated with liver transplantation and CC requires surgical intervention.Since IgG4-SC was first described,it has become a third distinct clinical entity of sclerosing cholangitis.The aim of this review was to introduce the diagnostic methods for IgG4-SC.IgG4-SC should be carefully diagnosed based on a combination of characteristic clinical,serological,morphological,and histopathological features after cholangiographic classification and targeting of a disease for differential diagnosis.When intrapancreatic stenosis is detected,pancreatic cancer or CC should be ruled out.If multiple intrahepatic stenoses are evident,PSC should be distinguished on the basis of cholangiographic findings and liver biopsy with IgG4 immunostaining.Associated inflammatory bowel disease is suggestive of PSC.If stenosis is demonstrated in the hepatic hilar region,CC should be discriminated by ultrasonography,intraductal ultrasonography,bile duct biopsy,and a higher cutoff serum IgG4 level of 182 mg/dL.
基金Supported by Partners Seeking a Cure and a VIDI grant from the Netherlands Organization for Scientific Research,No.016.136.308(to Weersma RK)
文摘AIM:To review the current literature for the specificclinical characteristics of inflammatory bowel disease(IBD)associated with primary sclerosing cholangitis(PSC).METHODS:A systematical review for clinical characteristics of IBD in PSC was performed by conducting a broad search for"primary sclerosing cholangitis"in Pubmed."Clinical characteristics"were specified into five predefined subthemes:epidemiology of IBD in PSC,characteristics of IBD in PSC(i.e.,location,disease behavior),risk of colorectal cancer development,IBD recurrence and de novo disease after liver transplantation for PSC,and safety and complications after proctocolectomy with ileal pouchanal anastomosis.Papers were selected for inclusion based on their relevance to the subthemes,and were reviewed by two independent reviewers.Only full papers relevant to PSC-IBD were included.Additionally the references of recent reviews for PSC(<5 years old)were scrutinized for relevant articles.RESULTS:Initial literature search for PSC yielded 4704results.After careful review 65 papers,comprising a total of 11406 PSC-IBD patients,were selected and divided according to subtheme.Four manuscripts overlapped and were included in two subthemes.Prevalence of IBD in PSC shows a large variance,ranging from 46.5%to 98.7%with ulcerative colitis(UC)being the most common type(>75%).The highest IBD rates in PSC are found in papers reviewing both endoscopic and histological data for IBD diagnosis.Although IBD in PSC is found to be a quiescent disease,pancolitis occurs often,with rates varying from 35%to 95%.Both backwash ileitis and rectal sparing are observed infrequently.The development of dysplasia or colorectal carcinoma is increased in PSC-IBD;the cumulative 10 years risk varying between 0%and11%.Exacerbation of IBD is common after liver transplantation for PSC and de novo disease is seen in1.3%to 31.3%of PSC-IBD patients.The risk for development of pouchitis in PSC-IBD is found to besignificant,affecting 13.8%to 90%of the patients after proctocolectomy with ileo anal-pouch anastomosis.CONCLUSION:IBD in primary sclerosing cholangitis represents a distinct phenotype that differs from UC and Crohn’s disease and therefore requires specialized management.
文摘Primary sclerosing cholangitis (PSC) is a chronic progressive inflammatory disease affecting the bile ducts, leading to f ibrosis and eventually cirrhosis in most patients. Its etiology is unknown and so far no effective medical therapy is available. Liver transplantation (LTX) is the only curative treatment and at present PSC is the main indication for LTX in the Scandinavian countries. Close to half of the PSC patients experience one or more episodes of acute cellular rejection (ACR) following transplantation and approximately 1/5 of the transplanted patients develop recurrent disease in the graft. In addition, some reports indicate that ACR early after LTX for PSC can infl uence the risk for recurrent disease. For these important post-transplantation entities affecting PSC patients, we have reviewed the current literature on epidemiology, pathogenesis, treatment and the possible infl uence of rejection on the risk of recurrent disease in the allograft.
基金Supported by Career Development Fellowship of the National Health and Medical Research Council of Australia,to Leong R
文摘To examine and evaluate recent evidence regarding the epidemiology, pathogenesis and management of colorectal cancer(CRC) development in inflammatory bowel disease(IBD)-primary sclerosing cholangitis(PSC) patients. Using the PubMed database, a literature search was conducted for relevant articles in English from the past 10 years. Relevant studies investigating PSC as a risk factor for CRC in IBD in the context of incidence and prevalence, pathogenesis, prevention and prognosis were included in this review. Recent evidence increasingly points to PSC as a significant risk factor in the development of CRC in patients with concomitant IBD. PSC may be an important risk factor for CRC in different populations worldwide. The mechanism for this increase in risk is still unclear. The efficacy of UDCA as a chemopreventive agent remains controversial. Liver transplantation does not halt the development of CRC, although there is not enough evidence to suggest that it is associated with increased incidence of CRC. While routine colonoscopic surveillance should be performed in patients with concurrent PSC and IBD, more high-level evidence is required to support the benefits of the procedure. While many new developments have taken place in the last decade, the pathogenesis and optimal management of CRC development in IBD-PSC patients remain unclear.
文摘Sclerosing cholangitis(SC)is a rarely reported morbidity secondary to transcatheter arterial chemoembolization(TACE)with bleomycin-iodinated oil(BIO)for liver cavernous hemangioma(LCH).This report retrospectively evaluated the diagnostic and therapeutic course of a patient with LDH who presented obstructive jaundice 6 years after TACE with BIO.Preoperative imaging identified a suspected malignant biliary stricture located at the convergence of the left and right hepatic ducts.Operative exploration demonstrated a full-thickness sclerosis of the hilar bile duct with right hepatic duct stricture and right lobe atrophy.Radical hepatic hilar resection with right-side hemihepatectomy and Roux-en-Y hepaticojejunostomy was performed because hilar cancer could not be excluded on frozen biopsy.Pathological results showed chronic pyogenic inflammation of the common and right hepatic ducts with SC in the portal area.Secondary SC is a long-term complication that may occur in LCH patients after TACE with BIO and must be differentiated from hilar malignancy.Hepatic duct plasty is a definitive but technically challenging treatment modality for secondary SC.
文摘Patients with primary sclerosing cholangitis(PSC) complicated by inflammatory bowel disease(IBD) represent a distinct subset of patients with unique characteristics,which have serious clinical implications.The aim of this literature review was to shed light to the obscure clinical and molecular aspects of the two diseases combined utilizing current data available and putting issues of diagnosis and treatment into perspective.The prevalence of IBD,mainly ulcerative colitis in PSC patients is estimated to be 21%-80%,dependent on screening programs and nationality.PSC-associated colitis is likely to be extensive,characterized by rectal sparing,backwash ileitis,and generally mild symptoms.It is also more likely to progress to colorectal malignancy,making it imperative for clinicians to maintain a high level of suspicion when tackling PSC patients.There is no optimal surveillance strategy but current guidelines advocate that colonoscopy is necessary at the time of PSC diagnosis with annual endoscopic follow-up.Random biopsies have been criticized and a shift towards targeted biopsies using chromoendoscopy,laser endomicroscopy and narrow-band imaging has been noted.Techniques directed towards genetic mutations instead of histological abnormalities hold promise for easier,more accurate diagnosis of dysplastic lesions.Chemopreventive measures against colorectal cancer have been sought in these patients.Ursodeoxycholic acid seemed promising at first but subsequent studies yielded conflicting results showing anticarcinogenic effects in low doses(8-15 mg/kg per day) and carcinogenic properties in high doses(15-30 mg/kg per day).
基金Supported by Ministry of Health of the Czech Republic,No.15-28064A
文摘To characterize the gut bacterial microbiota of patients with primary sclerosing cholangitis(PSC)and ulcerative colitis(UC).METHODSStool samples were collected and relevant clinical data obtained from 106 study participants,43 PSC patients with(n=32)or without(n=11)concomitant inflammatory bowel disease,32 UC patients,and 31 healthy controls.The V3 and V4 regions of the 16S ribosomal RNA gene were sequenced on Illumina MiSeq platform to cover low taxonomic levels.Data were further processed in QIIME employing MaAsLin and LEfSe tools for analysis of the output data.RESULTSMicrobial profiles in both PSC and UC were characterized by low bacterial diversity and significant change in global microbial composition.Rothia,Enterococcus,Streptococcus,Veillonella,and three other genera were markedly overrepresented in PSC regardless of concomitant inflammatory bowel disease(IBD).Rothia,Veillonella and Streptococcus were tracked to the species level to identify Rothia mucilaginosa,Streptococcus infantus,S.alactolyticus,and S.equi along with Veillonella parvula and V.dispar.PSC was further characterized by decreased abundance of Adlercreutzia equolifaciens and Prevotella copri.Decrease in genus Phascolarctobacterium was linked to presence of colonic inflammation regardless of IBD phenotype.Akkermansia muciniphila,Butyricicoccus pullicaecorum and Clostridium colinum were decreased in UC along with genus Roseburia.Low levels of serum albumin were significantly correlated with enrichment of order Actinomycetales.CONCLUSIONPSC is associated with specific gut microbes independently of concomitant IBD and several bacterial taxa clearly distinguish IBD phenotypes(PSC-IBD and UC).
