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
BACKGROUND Ovarian sclerosing stromal tumors(OSSTs)are found most commonly in females at 20-30 years of age.They can also occur at any point during pre-puberty,puberty,or menopause.Clinical manifestations of OSSTs inc...BACKGROUND Ovarian sclerosing stromal tumors(OSSTs)are found most commonly in females at 20-30 years of age.They can also occur at any point during pre-puberty,puberty,or menopause.Clinical manifestations of OSSTs include abdominal pain,an abdominal mass,menstrual abnormalities,and infertility.Infrequently,patients will experience androgen-related manifestations of masculinization,such as increased hair,acne,or a low voice.Diagnosis must be confirmed by immunohistochemical analysis of the tissue as clinical symptoms and imaging studies are unreliable.CASE SUMMARY A 14-year-old female presented with amenorrhea.After a thorough medical examination,endocrine and tumor markers analysis,and imaging,a pelvic mass was discovered.The patient also exhibited endocrine dysfunction but was not positive for any tumor markers.The patient underwent surgery to remove the ovarian tumor.Immunohistochemical analysis of the resected specimen indicated an OSST.During the postoperative follow-up,the patient had attained menarche.CONCLUSION This case’s clinical manifestation of endocrine dysfunction due to OSST provides new insights that will assist clinicians in the diagnosis and treatment of this common tumor.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
To investigate the histological,immunohistochemical,and molecular characteristics of sclerosing epithelioid fibrosarcoma(SEF),a rare intracranial lesion,we analyzed the clinical,pathological,and molecular features of ...To investigate the histological,immunohistochemical,and molecular characteristics of sclerosing epithelioid fibrosarcoma(SEF),a rare intracranial lesion,we analyzed the clinical,pathological,and molecular features of a group of cases involving both intracranial(case 1)and extracranial(cases 2,3,and 4)soft tissue tumors.These tumors were located in the bilateral parietal sinuses(one of four cases;25%),thoracic vertebrae(1 of 4 cases;25%),mediastinum(1 of 4 cases;25%),and parathyroid soft tissue(1 of 4 cases;25%).Microscopically,tumor cells were observed in both sparse and dense areas,arranged in sheets,cords,and a fusiform braided pattern,all within a dense sclerotic matrix.All four patients exhibited strong and diffuse positivity for MUC4.Cases 1 and 2 harbored an EWSR1–CREB3L1 fusion;case 3 had a CPSF6–ITPR2 fusion;and case 4 exhibited multiple fusion genes,including FUS–CREB3L2,KDM5A–ERC1,AKAP8L–BRD4,and ATF2–CHN1.Methylation analysis indicated that all cases clustered within the SEF spectrum.Copy number variation analysis revealed deletions in chromosomal arms 11p and 22q across all cases.The morphologies of intracranial and extracranial SEF were found to be similar.Although MUC4 serves as a reliable marker for diagnosing SEF,its molecular findings remain nonspecific.Methylation clustering and aneuploidy score assessment should be considered as additional tools to assist in diagnosis and prognostic monitoring.展开更多
Primary sclerosing cholangitis(PSC)is an autoimmune cholangiopathy characterized by chronic inflammation of the biliary epithelium and periductal fibrosis,with no curative treatment available,and liver transplantation...Primary sclerosing cholangitis(PSC)is an autoimmune cholangiopathy characterized by chronic inflammation of the biliary epithelium and periductal fibrosis,with no curative treatment available,and liver transplantation is inevitable for end-stage patients.Human placentalmesenchymal stem cell(hpMSC)-derived exosomes have demonstrated the ability to prevent fibrosis,inhibit collagen production and possess immunomodulatory properties in autoimmune liver disease.Here,we prepared hpMSC-derived exosomes(Exo^(MSC))and further investigated the anti-fibrotic effects and detailed mechanism on PSC based on Mdr2^(−/−)mice and multicellular organoids established from PSC patients.The results showed that Exo^(MSC) ameliorated liver fibrosis in Mdr2^(−/−)mice with significant collagen reduction in the preductal area where Th17 differentiation was inhibited as demonstrated by RNAseq analysis,and the percentage of CD4+IL-17A+T cells was reduced both in Exo^(MSC)-treated Mdr2^(−/−)mice(Mdr2^(−/−)-Exo)in vivo and Exo^(MSC)-treated Th17 differentiation progressed in vitro.Furthermore,Exo^(MSC) improved the hypersecretory phenotype and intercellular interactions in the hepatic Th17 microenvironment by regulating PERK/CHOP signaling as supported by multicellular organoids.Thus,our data demonstrate the antifibrosis effect of Exo^(MSC) in PSC disease by inhibiting Th17 differentiation,and ameliorating the Th17-induced microenvironment,indicating the promising potential therapeutic role of Exo^(MSC) in liver fibrosis of PSC or Th17-related diseases.展开更多
IgG4-related sclerosing cholangitis(IgG4-SC)is an inflammatory disease that leads to bile duct stricture,characterized by the infiltration of IgG4-positive plasma cells into the bile duct wall,thickening of the bile d...IgG4-related sclerosing cholangitis(IgG4-SC)is an inflammatory disease that leads to bile duct stricture,characterized by the infiltration of IgG4-positive plasma cells into the bile duct wall,thickening of the bile duct wall,and narrowing of the lumen.The differential diagnosis of IgG4-SC mainly includes primary sclerosing cholangitis,cholangiocarcinoma,and pancreatic cancer.IgG4-SC is often associated with autoimmune pancreatitis and can be accurately diagnosed based on clinical diagnostic criteria.However,isolated IgG4-SC is difficult to distinguish from biliary tumors.Given the significant differences in biological behavior,treatment,and prognosis between these diseases,accurately identifying isolated IgG4-SC has very important clinical significance.展开更多
BACKGROUND The complexity of immunoglobulin G4(IgG4)-related diseases and their potential connection to hematologic malignancies remains unclear.This article provided a review of the diagnosis and treatment of a patie...BACKGROUND The complexity of immunoglobulin G4(IgG4)-related diseases and their potential connection to hematologic malignancies remains unclear.This article provided a review of the diagnosis and treatment of a patient with IgG4-related sclerosing cholangitis(SC)and essential thrombocythemia(ET),along with an analysis of relevant literature to enhance comprehension of this disease.CASE SUMMARY A 56-year-old male was admitted to two hospitals with deteriorating jaundice and pruritus prior to hospitalization.Beyond our expectations,the patient was first diagnosed with IgG4-SC and ET with the Janus kinase 2 V617F mutation.Interestingly,the administration of acetate prednisone significantly resulted in improvements in both IgG4-SC and ET.Clinicians need to pay attention to immune disorders and inflammation as they contribute to the development of various disease phenotypes.CONCLUSION When IgG4-SC is suspected without histopathological evidence,diagnostic therapy and long-term regular follow-up can lead to positive treatment outcomes.Clinicians should be mindful of the potential presence of concurrent hematologic diseases in patients with immune disorders.展开更多
BACKGROUND Chronic hepatitis C virus(HCV)infection is a major global health concern that leads to liver fibrosis,cirrhosis,and cancer.Regimens containing direct-acting antivirals(DAAs)have become the mainstay of HCV t...BACKGROUND Chronic hepatitis C virus(HCV)infection is a major global health concern that leads to liver fibrosis,cirrhosis,and cancer.Regimens containing direct-acting antivirals(DAAs)have become the mainstay of HCV treatment,achieving a high sustained virological response(SVR)with minimal adverse events.CASE SUMMARY A 74-year-old woman with chronic HCV infection was treated with the DAAs ledipasvir,and sofosbuvir for 12 wk and achieved SVR.Twenty-four weeks after treatment completion,the liver enzyme and serum IgG levels increased,and antinuclear antibody became positive without HCV viremia,suggesting the development of autoimmune hepatitis(AIH).After liver biopsy indicated AIH,a definite AIH diagnosis was made and prednisolone was initiated.The treatment was effective,and the liver enzyme and serum IgG levels normalized.However,multiple strictures of the intrahepatic and extrahepatic bile ducts with dilatation of the peripheral bile ducts appeared on magnetic resonance cholangiopancreatography after 3 years of achieving SVR,which were consistent with primary sclerosing cholangitis.CONCLUSION The potential risk of developing autoimmune liver diseases after DAA treatment should be considered.展开更多
BACKGROUND A sclerosing epithelioid fibrosarcoma(SEF)is a rare malignant fibroblastic soft tissue tumor that rarely occurs in intra-abdominal organs.A case of a SEF in the pancreatic head is reported herein,including ...BACKGROUND A sclerosing epithelioid fibrosarcoma(SEF)is a rare malignant fibroblastic soft tissue tumor that rarely occurs in intra-abdominal organs.A case of a SEF in the pancreatic head is reported herein,including its clinical manifestations,preoperative imaging features,gross specimen and pathological findings.CASE SUMMARY A 33-year-old male patient was admitted to Peking Union Medical College Hospital in December 2023 due to a one-year history of intermittent upper abdominal pain and the discovery of a pancreatic mass.The patient underwent an enhanced computed tomography scan of the abdomen,which revealed a welldefined,round mass with clear borders and calcifications in the pancreatic head.The mass exhibited progressive,uneven mild enhancement,measuring approximately 6.6 cm×6.3 cm.The patient underwent laparoscopic pylorus-preserving pancreaticoduodenectomy.Postoperative pathological examination revealed that the lesion was consistent with a SEF.At the 3-month postoperative follow-up,the patient did not report any short-term complications,and there were no signs of tumor recurrence.CONCLUSION SEFs are rare malignant fibrous soft tissue tumors.SEFs rarely develop in the pancreas,and its preoperative diagnosis depends on imaging findings,with confirmation depending on pathological examination and immunohistochemistry.Currently,only four cases of pancreatic SEF have been reported in studies written in English.This case is the first reported case of a pancreatic SEF by a clinical physician.展开更多
Rationale:Subacute sclerosing panencephalitis(SSPE)is a progressive neurological disorder caused by persistent measles virus infection.SSPE predominantly affects children and adolescents.The symptoms usually develop 6...Rationale:Subacute sclerosing panencephalitis(SSPE)is a progressive neurological disorder caused by persistent measles virus infection.SSPE predominantly affects children and adolescents.The symptoms usually develop 6-15 years after measles infection and ultimately leading to death in many cases.Patient concerns:Patient 1 presented with cognitive decline and myoclonus and the Patient 2 presented with diminution of vision with myoclonic jerks.Diagnosis:Based on the clinical features with a characteristic electroencephalogram pattern and the presence of a high titer of anti-measles IgG in serum and cerebrospinal fluid,these patients were diagnosed as SSPE.Interventions:Antiepileptics were started for controlling myoclonus along with supportive treatment.Outcomes:Both patients were discharged on antiepileptics and supportive care.Lessons:Whenever there are unusual clinical manifestations with unknown vaccination status,SSPE can be suspected and the cerebrospinal fluid should be examined for anti-measles antibodies.Our case study also highlights the importance of universal coverage of measles vaccination.To reduce the incidence of measles and associated deaths,it is important to maintain a high level of immunization coverage for the measles vaccine and to strengthen all the integral components of the national immunization program.展开更多
BACKGROUND Primary sclerosing cholangitis(PSC)is characterized by chronic inflammation and it predisposes to cholangiocarcinoma due to lack of effective treatment options.Recombinant adeno-associated virus(rAAV)provid...BACKGROUND Primary sclerosing cholangitis(PSC)is characterized by chronic inflammation and it predisposes to cholangiocarcinoma due to lack of effective treatment options.Recombinant adeno-associated virus(rAAV)provides a promising platform for gene therapy on such kinds of diseases.A microRNA(miRNA)let-7a has been reported to be associated with the progress of PSC but the potential therapeutic implication of inhibition of let-7a on PSC has not been evaluated.AIM To investigate the therapeutic effects of inhibition of a miRNA let-7a transferred by recombinant adeno-associated virus 8(rAAV8)on a xenobiotic-induced mouse model of sclerosing cholangitis.METHODS A xenobiotic-induced mouse model of sclerosing cholangitis was induced by 0.1% 3,5-Diethoxycarbonyl-1,4-Dihydrocollidine(DDC)feeding for 2 wk or 6 wk.A single dose of rAAV8-mediated anti-let-7a-5p sponges or scramble control was injected in vivo into mice onset of DDC feeding.Upon sacrifice,the liver and the serum were collected from each mouse.The hepatobiliary injuries,hepatic inflammation and fibrosis were evaluated.The targets of let-7a-5p and downstream molecule NF-κB were detected using Western blot.RESULTS rAAV8-mediated anti-let-7a-5p sponges can depress the expression of let-7a-5p in mice after DDC feeding for 2 wk or 6 wk.The reduced expression of let-7a-5p can alleviate hepato-biliary injuries indicated by serum markers,and prevent the proliferation of cholangiocytes and biliary fibrosis.Furthermore,inhibition of let-7a mediated by rAAV8 can increase the expression of potential target molecules such as suppressor of cytokine signaling 1 and Dectin1,which consequently inhibit of NF-κB-mediated hepatic inflammation.CONCLUSION Our study demonstrates that a rAAV8 vector designed for liver-specific inhibition of let-7a-5p can potently ameliorate symptoms in a xenobiotic-induced mouse model of sclerosing cholangitis,which provides a possible clinical translation of PSC of human.展开更多
BACKGROUND The purpose of this case report is to describe a case of multiple intrahepatic artery aneurysms during treatment for IgG4-related sclerosing cholangitis(IgG4-SC)and to provide information for daily practice...BACKGROUND The purpose of this case report is to describe a case of multiple intrahepatic artery aneurysms during treatment for IgG4-related sclerosing cholangitis(IgG4-SC)and to provide information for daily practice.CASE SUMMARY A 64-year-old Japanese woman was diagnosed with IgG4-SC five years prior and was receiving maintenance treatment with prednisolone 7.5-10 mg/day.She developed abdominal pain and a sudden onset of black stool and was admitted to our hospital.Abdominal contrast-enhanced computed tomography(CT)and ultra-sonography(US)revealed multiple intrahepatic artery aneurysms that developed during the treatment for IgG4-SC.Emergency transarterial embolization for mu-ltiple hepatic artery aneurysms was performed.Hepatic artery aneurysms disappeared on contrast-enhanced CT and US,the progression of anemia ceased,and the melena resolved.Thus,hemostasis was achieved.CONCLUSION Hepatic artery aneurysms should be considered poor prognostic complications of IgG4-SC.展开更多
BACKGROUND Ulcerative colitis(UC)with concomitant primary sclerosing cholangitis(PSC)represents a distinct disease entity(PSC-UC).Mayo endoscopic subscore(MES)is a standard tool for assessing disease activity in UC bu...BACKGROUND Ulcerative colitis(UC)with concomitant primary sclerosing cholangitis(PSC)represents a distinct disease entity(PSC-UC).Mayo endoscopic subscore(MES)is a standard tool for assessing disease activity in UC but its relevance in PSC-UC remains unclear.AIM To assess the accuracy of MES in UC and PSC-UC patients,we performed histological scoring using Nancy histological index(NHI).METHODS MES was assessed in 30 PSC-UC and 29 UC adult patients during endoscopy.NHI and inflammation were evaluated in biopsies from the cecum,rectum,and terminal ileum.In addition,perinuclear anti-neutrophil cytoplasmic antibodies,fecal calprotectin,body mass index,and other relevant clinical characteristics were collected.RESULTS The median MES and NHI were similar for UC patients(MES grade 2 and NHI grade 2 in the rectum)but were different for PSC-UC patients(MES grade 0 and NHI grade 2 in the cecum).There was a correlation between MES and NHI for UC patients(Spearman's r=0.40,P=0.029)but not for PSC-UC patients.Histopathological examination revealed persistent microscopic inflammation in 88%of PSC-UC patients with MES grade 0(46%of all PSC-UC patients).Moreover,MES overestimated the severity of active inflammation in an additional 11%of PSCUC patients.CONCLUSION MES insufficiently identifies microscopic inflammation in PSC-UC.This indicates that histological evaluation should become a routine procedure of the diagnostic and grading system in both PSC-UC and PSC.展开更多
Sclerosing mucoepidermoid thyroid cancer (SMECE) is a rare entity with less than 100 cases reported in the literature. Previously considered to have an indolent course, however, recent evidence has reported an aggress...Sclerosing mucoepidermoid thyroid cancer (SMECE) is a rare entity with less than 100 cases reported in the literature. Previously considered to have an indolent course, however, recent evidence has reported an aggressive nature ranging from local invasion to distant metastasis. We present a 66-year-old Caucasian female with SMECE who initially presented neck compressive symptoms. A thyroid ultrasound (US) revealed a solid hypoechoic mass replacing the left thyroid lobe. Fine needle aspiration cytology (FNAC) of the nodule resulted in suspicion of Papillary Thyroid Cancer, Bethesda category 5. The patient underwent total thyroidectomy and surgical pathology showed SMECE. Post-therapy whole-body scan following treatment with 150 mCi I-131 showed no residual or metastatic disease. SMECE is more common in females, between the third to eighth decade of life. Preoperative diagnosis may not be accurate given variable cytopathologic features. Differential diagnoses include primary squamous cell carcinoma of the thyroid, squamous differentiation of other thyroid malignancies, anaplastic thyroid cancer and nodular sclerosing variety of Hodgkin’s lymphoma. Due to its rarity, treatment of SMECE has ranged from thyroid surgery without or with radioactive iodine therapy, to surgery and external beam radiation and even chemotherapy.展开更多
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
Based on histological and immunohistochemical exami- nation of various organs of patients with autoimmune pancreatitis （AIP）, a novel clinicopathological entity of IgG4-related sclerosing disease has been proposed. ...Based on histological and immunohistochemical exami- nation of various organs of patients with autoimmune pancreatitis （AIP）, a novel clinicopathological entity of IgG4-related sclerosing disease has been proposed. This is a systemic disease that is characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration of various organs. Clinical manifestations are apparent in the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prosrate, in which tissue fibrosis with obliterative phlebitis is pathologically induced. AlP is not simply pancreatitis but, in fact, is a pancreatic disease indicative of IgG4- related sclerosing diseases. This disease includes AlP, sclerosing cholangitis, cholecystitis, sialadenitis, retro-peritoneal fibrosis, tubulointerstitial nephritis, interstitial pneumonia, prostatitis, inflammatory pseudotumor and lymphadenopathy, all IgG4-related. Most IgG4-related sclerosing diseases have been found to be associated with AlP, but also those without pancreatic involvement have been reported. In some cases, only one or two organs are clinically involved, while in others, three or four organs are affected. The disease occurs predominantly in older men and responds well to steroid therapy. Serum IgG4 levels and immunos-taining with anti-IgG4 antibody are useful in making the diagnosis. Since malignant tumors are frequently suspected on initial presentation, IgG4-related sclerosing disease should be considered in the differential diagnosis to avoid unnecessary surgery.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
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
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BACKGROUND Ovarian sclerosing stromal tumors(OSSTs)are found most commonly in females at 20-30 years of age.They can also occur at any point during pre-puberty,puberty,or menopause.Clinical manifestations of OSSTs include abdominal pain,an abdominal mass,menstrual abnormalities,and infertility.Infrequently,patients will experience androgen-related manifestations of masculinization,such as increased hair,acne,or a low voice.Diagnosis must be confirmed by immunohistochemical analysis of the tissue as clinical symptoms and imaging studies are unreliable.CASE SUMMARY A 14-year-old female presented with amenorrhea.After a thorough medical examination,endocrine and tumor markers analysis,and imaging,a pelvic mass was discovered.The patient also exhibited endocrine dysfunction but was not positive for any tumor markers.The patient underwent surgery to remove the ovarian tumor.Immunohistochemical analysis of the resected specimen indicated an OSST.During the postoperative follow-up,the patient had attained menarche.CONCLUSION This case’s clinical manifestation of endocrine dysfunction due to OSST provides new insights that will assist clinicians in the diagnosis and treatment of this common tumor.
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.
基金supported by grants from the National Natural Science Foundation of China(No.82273082)Chongqing Technical Innovation and Application Development Key Project(No.cstc2021jscx-cylhX0005)。
文摘To investigate the histological,immunohistochemical,and molecular characteristics of sclerosing epithelioid fibrosarcoma(SEF),a rare intracranial lesion,we analyzed the clinical,pathological,and molecular features of a group of cases involving both intracranial(case 1)and extracranial(cases 2,3,and 4)soft tissue tumors.These tumors were located in the bilateral parietal sinuses(one of four cases;25%),thoracic vertebrae(1 of 4 cases;25%),mediastinum(1 of 4 cases;25%),and parathyroid soft tissue(1 of 4 cases;25%).Microscopically,tumor cells were observed in both sparse and dense areas,arranged in sheets,cords,and a fusiform braided pattern,all within a dense sclerotic matrix.All four patients exhibited strong and diffuse positivity for MUC4.Cases 1 and 2 harbored an EWSR1–CREB3L1 fusion;case 3 had a CPSF6–ITPR2 fusion;and case 4 exhibited multiple fusion genes,including FUS–CREB3L2,KDM5A–ERC1,AKAP8L–BRD4,and ATF2–CHN1.Methylation analysis indicated that all cases clustered within the SEF spectrum.Copy number variation analysis revealed deletions in chromosomal arms 11p and 22q across all cases.The morphologies of intracranial and extracranial SEF were found to be similar.Although MUC4 serves as a reliable marker for diagnosing SEF,its molecular findings remain nonspecific.Methylation clustering and aneuploidy score assessment should be considered as additional tools to assist in diagnosis and prognostic monitoring.
基金supported by grants for National Key Research and Development Program of China(No.2020YFA0113003)Key Research and Development Project of Zhejiang Province(No.2023C03046)+1 种基金Fundamental Research Funds for the Central Universities(No.2022ZFJH003)Research Project of Jinan Microecological Biomedicine Shandong Laboratory(No.JNL-2022026C,JNL-2023003C).
文摘Primary sclerosing cholangitis(PSC)is an autoimmune cholangiopathy characterized by chronic inflammation of the biliary epithelium and periductal fibrosis,with no curative treatment available,and liver transplantation is inevitable for end-stage patients.Human placentalmesenchymal stem cell(hpMSC)-derived exosomes have demonstrated the ability to prevent fibrosis,inhibit collagen production and possess immunomodulatory properties in autoimmune liver disease.Here,we prepared hpMSC-derived exosomes(Exo^(MSC))and further investigated the anti-fibrotic effects and detailed mechanism on PSC based on Mdr2^(−/−)mice and multicellular organoids established from PSC patients.The results showed that Exo^(MSC) ameliorated liver fibrosis in Mdr2^(−/−)mice with significant collagen reduction in the preductal area where Th17 differentiation was inhibited as demonstrated by RNAseq analysis,and the percentage of CD4+IL-17A+T cells was reduced both in Exo^(MSC)-treated Mdr2^(−/−)mice(Mdr2^(−/−)-Exo)in vivo and Exo^(MSC)-treated Th17 differentiation progressed in vitro.Furthermore,Exo^(MSC) improved the hypersecretory phenotype and intercellular interactions in the hepatic Th17 microenvironment by regulating PERK/CHOP signaling as supported by multicellular organoids.Thus,our data demonstrate the antifibrosis effect of Exo^(MSC) in PSC disease by inhibiting Th17 differentiation,and ameliorating the Th17-induced microenvironment,indicating the promising potential therapeutic role of Exo^(MSC) in liver fibrosis of PSC or Th17-related diseases.
基金Supported by The Science and Technology Research Foundation of Guizhou Province,and Zunyi City,No.QKHJC-ZK(2022)YB642,No.ZSKH·HZ(2022)344,and No.gzwjkj2021-071The WBE Liver Fibrosis Foundation,No.CFHPC2025028The Beijing Gandan Phase Mutual Public Welfare Fund for Artificial Liver Project,No.iGandanF-1082024-Rgg018.
文摘IgG4-related sclerosing cholangitis(IgG4-SC)is an inflammatory disease that leads to bile duct stricture,characterized by the infiltration of IgG4-positive plasma cells into the bile duct wall,thickening of the bile duct wall,and narrowing of the lumen.The differential diagnosis of IgG4-SC mainly includes primary sclerosing cholangitis,cholangiocarcinoma,and pancreatic cancer.IgG4-SC is often associated with autoimmune pancreatitis and can be accurately diagnosed based on clinical diagnostic criteria.However,isolated IgG4-SC is difficult to distinguish from biliary tumors.Given the significant differences in biological behavior,treatment,and prognosis between these diseases,accurately identifying isolated IgG4-SC has very important clinical significance.
基金Natural Science Foundation of Hebei Province,No.H2023206042。
文摘BACKGROUND The complexity of immunoglobulin G4(IgG4)-related diseases and their potential connection to hematologic malignancies remains unclear.This article provided a review of the diagnosis and treatment of a patient with IgG4-related sclerosing cholangitis(SC)and essential thrombocythemia(ET),along with an analysis of relevant literature to enhance comprehension of this disease.CASE SUMMARY A 56-year-old male was admitted to two hospitals with deteriorating jaundice and pruritus prior to hospitalization.Beyond our expectations,the patient was first diagnosed with IgG4-SC and ET with the Janus kinase 2 V617F mutation.Interestingly,the administration of acetate prednisone significantly resulted in improvements in both IgG4-SC and ET.Clinicians need to pay attention to immune disorders and inflammation as they contribute to the development of various disease phenotypes.CONCLUSION When IgG4-SC is suspected without histopathological evidence,diagnostic therapy and long-term regular follow-up can lead to positive treatment outcomes.Clinicians should be mindful of the potential presence of concurrent hematologic diseases in patients with immune disorders.
文摘BACKGROUND Chronic hepatitis C virus(HCV)infection is a major global health concern that leads to liver fibrosis,cirrhosis,and cancer.Regimens containing direct-acting antivirals(DAAs)have become the mainstay of HCV treatment,achieving a high sustained virological response(SVR)with minimal adverse events.CASE SUMMARY A 74-year-old woman with chronic HCV infection was treated with the DAAs ledipasvir,and sofosbuvir for 12 wk and achieved SVR.Twenty-four weeks after treatment completion,the liver enzyme and serum IgG levels increased,and antinuclear antibody became positive without HCV viremia,suggesting the development of autoimmune hepatitis(AIH).After liver biopsy indicated AIH,a definite AIH diagnosis was made and prednisolone was initiated.The treatment was effective,and the liver enzyme and serum IgG levels normalized.However,multiple strictures of the intrahepatic and extrahepatic bile ducts with dilatation of the peripheral bile ducts appeared on magnetic resonance cholangiopancreatography after 3 years of achieving SVR,which were consistent with primary sclerosing cholangitis.CONCLUSION The potential risk of developing autoimmune liver diseases after DAA treatment should be considered.
基金Supported by National High Level Hospital Clinical Research Funding,No.2022-PUMCH-B-003National Multidisciplinary Cooperative Diagnosis and Treatment Capacity Building Project for Major Diseases.
文摘BACKGROUND A sclerosing epithelioid fibrosarcoma(SEF)is a rare malignant fibroblastic soft tissue tumor that rarely occurs in intra-abdominal organs.A case of a SEF in the pancreatic head is reported herein,including its clinical manifestations,preoperative imaging features,gross specimen and pathological findings.CASE SUMMARY A 33-year-old male patient was admitted to Peking Union Medical College Hospital in December 2023 due to a one-year history of intermittent upper abdominal pain and the discovery of a pancreatic mass.The patient underwent an enhanced computed tomography scan of the abdomen,which revealed a welldefined,round mass with clear borders and calcifications in the pancreatic head.The mass exhibited progressive,uneven mild enhancement,measuring approximately 6.6 cm×6.3 cm.The patient underwent laparoscopic pylorus-preserving pancreaticoduodenectomy.Postoperative pathological examination revealed that the lesion was consistent with a SEF.At the 3-month postoperative follow-up,the patient did not report any short-term complications,and there were no signs of tumor recurrence.CONCLUSION SEFs are rare malignant fibrous soft tissue tumors.SEFs rarely develop in the pancreas,and its preoperative diagnosis depends on imaging findings,with confirmation depending on pathological examination and immunohistochemistry.Currently,only four cases of pancreatic SEF have been reported in studies written in English.This case is the first reported case of a pancreatic SEF by a clinical physician.
文摘Rationale:Subacute sclerosing panencephalitis(SSPE)is a progressive neurological disorder caused by persistent measles virus infection.SSPE predominantly affects children and adolescents.The symptoms usually develop 6-15 years after measles infection and ultimately leading to death in many cases.Patient concerns:Patient 1 presented with cognitive decline and myoclonus and the Patient 2 presented with diminution of vision with myoclonic jerks.Diagnosis:Based on the clinical features with a characteristic electroencephalogram pattern and the presence of a high titer of anti-measles IgG in serum and cerebrospinal fluid,these patients were diagnosed as SSPE.Interventions:Antiepileptics were started for controlling myoclonus along with supportive treatment.Outcomes:Both patients were discharged on antiepileptics and supportive care.Lessons:Whenever there are unusual clinical manifestations with unknown vaccination status,SSPE can be suspected and the cerebrospinal fluid should be examined for anti-measles antibodies.Our case study also highlights the importance of universal coverage of measles vaccination.To reduce the incidence of measles and associated deaths,it is important to maintain a high level of immunization coverage for the measles vaccine and to strengthen all the integral components of the national immunization program.
基金Supported by the National Natural Science Foundation of China,No.82172297Natural Science Foundation of Jiangsu Province of China,No.BK20211346 and No.BK20201011+1 种基金Natural Science Foundation of Jiangsu Higher Education Institutions of China,No.22KJA310007Xuzhou Science and Technology Project,No.KC22055.
文摘BACKGROUND Primary sclerosing cholangitis(PSC)is characterized by chronic inflammation and it predisposes to cholangiocarcinoma due to lack of effective treatment options.Recombinant adeno-associated virus(rAAV)provides a promising platform for gene therapy on such kinds of diseases.A microRNA(miRNA)let-7a has been reported to be associated with the progress of PSC but the potential therapeutic implication of inhibition of let-7a on PSC has not been evaluated.AIM To investigate the therapeutic effects of inhibition of a miRNA let-7a transferred by recombinant adeno-associated virus 8(rAAV8)on a xenobiotic-induced mouse model of sclerosing cholangitis.METHODS A xenobiotic-induced mouse model of sclerosing cholangitis was induced by 0.1% 3,5-Diethoxycarbonyl-1,4-Dihydrocollidine(DDC)feeding for 2 wk or 6 wk.A single dose of rAAV8-mediated anti-let-7a-5p sponges or scramble control was injected in vivo into mice onset of DDC feeding.Upon sacrifice,the liver and the serum were collected from each mouse.The hepatobiliary injuries,hepatic inflammation and fibrosis were evaluated.The targets of let-7a-5p and downstream molecule NF-κB were detected using Western blot.RESULTS rAAV8-mediated anti-let-7a-5p sponges can depress the expression of let-7a-5p in mice after DDC feeding for 2 wk or 6 wk.The reduced expression of let-7a-5p can alleviate hepato-biliary injuries indicated by serum markers,and prevent the proliferation of cholangiocytes and biliary fibrosis.Furthermore,inhibition of let-7a mediated by rAAV8 can increase the expression of potential target molecules such as suppressor of cytokine signaling 1 and Dectin1,which consequently inhibit of NF-κB-mediated hepatic inflammation.CONCLUSION Our study demonstrates that a rAAV8 vector designed for liver-specific inhibition of let-7a-5p can potently ameliorate symptoms in a xenobiotic-induced mouse model of sclerosing cholangitis,which provides a possible clinical translation of PSC of human.
文摘BACKGROUND The purpose of this case report is to describe a case of multiple intrahepatic artery aneurysms during treatment for IgG4-related sclerosing cholangitis(IgG4-SC)and to provide information for daily practice.CASE SUMMARY A 64-year-old Japanese woman was diagnosed with IgG4-SC five years prior and was receiving maintenance treatment with prednisolone 7.5-10 mg/day.She developed abdominal pain and a sudden onset of black stool and was admitted to our hospital.Abdominal contrast-enhanced computed tomography(CT)and ultra-sonography(US)revealed multiple intrahepatic artery aneurysms that developed during the treatment for IgG4-SC.Emergency transarterial embolization for mu-ltiple hepatic artery aneurysms was performed.Hepatic artery aneurysms disappeared on contrast-enhanced CT and US,the progression of anemia ceased,and the melena resolved.Thus,hemostasis was achieved.CONCLUSION Hepatic artery aneurysms should be considered poor prognostic complications of IgG4-SC.
基金Supported by Grant Agency of the Ministry of Health of the Czech Republic,No.NV17-31538AGrant Agency of the Czech Republic No.20-16520Y and No.21-21736SMinistry of Education,Youth and Sports of the Czech Republic Project,No.LX22NPO05102.
文摘BACKGROUND Ulcerative colitis(UC)with concomitant primary sclerosing cholangitis(PSC)represents a distinct disease entity(PSC-UC).Mayo endoscopic subscore(MES)is a standard tool for assessing disease activity in UC but its relevance in PSC-UC remains unclear.AIM To assess the accuracy of MES in UC and PSC-UC patients,we performed histological scoring using Nancy histological index(NHI).METHODS MES was assessed in 30 PSC-UC and 29 UC adult patients during endoscopy.NHI and inflammation were evaluated in biopsies from the cecum,rectum,and terminal ileum.In addition,perinuclear anti-neutrophil cytoplasmic antibodies,fecal calprotectin,body mass index,and other relevant clinical characteristics were collected.RESULTS The median MES and NHI were similar for UC patients(MES grade 2 and NHI grade 2 in the rectum)but were different for PSC-UC patients(MES grade 0 and NHI grade 2 in the cecum).There was a correlation between MES and NHI for UC patients(Spearman's r=0.40,P=0.029)but not for PSC-UC patients.Histopathological examination revealed persistent microscopic inflammation in 88%of PSC-UC patients with MES grade 0(46%of all PSC-UC patients).Moreover,MES overestimated the severity of active inflammation in an additional 11%of PSCUC patients.CONCLUSION MES insufficiently identifies microscopic inflammation in PSC-UC.This indicates that histological evaluation should become a routine procedure of the diagnostic and grading system in both PSC-UC and PSC.
文摘Sclerosing mucoepidermoid thyroid cancer (SMECE) is a rare entity with less than 100 cases reported in the literature. Previously considered to have an indolent course, however, recent evidence has reported an aggressive nature ranging from local invasion to distant metastasis. We present a 66-year-old Caucasian female with SMECE who initially presented neck compressive symptoms. A thyroid ultrasound (US) revealed a solid hypoechoic mass replacing the left thyroid lobe. Fine needle aspiration cytology (FNAC) of the nodule resulted in suspicion of Papillary Thyroid Cancer, Bethesda category 5. The patient underwent total thyroidectomy and surgical pathology showed SMECE. Post-therapy whole-body scan following treatment with 150 mCi I-131 showed no residual or metastatic disease. SMECE is more common in females, between the third to eighth decade of life. Preoperative diagnosis may not be accurate given variable cytopathologic features. Differential diagnoses include primary squamous cell carcinoma of the thyroid, squamous differentiation of other thyroid malignancies, anaplastic thyroid cancer and nodular sclerosing variety of Hodgkin’s lymphoma. Due to its rarity, treatment of SMECE has ranged from thyroid surgery without or with radioactive iodine therapy, to surgery and external beam radiation and even chemotherapy.
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Based on histological and immunohistochemical exami- nation of various organs of patients with autoimmune pancreatitis （AIP）, a novel clinicopathological entity of IgG4-related sclerosing disease has been proposed. This is a systemic disease that is characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration of various organs. Clinical manifestations are apparent in the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prosrate, in which tissue fibrosis with obliterative phlebitis is pathologically induced. AlP is not simply pancreatitis but, in fact, is a pancreatic disease indicative of IgG4- related sclerosing diseases. This disease includes AlP, sclerosing cholangitis, cholecystitis, sialadenitis, retro-peritoneal fibrosis, tubulointerstitial nephritis, interstitial pneumonia, prostatitis, inflammatory pseudotumor and lymphadenopathy, all IgG4-related. Most IgG4-related sclerosing diseases have been found to be associated with AlP, but also those without pancreatic involvement have been reported. In some cases, only one or two organs are clinically involved, while in others, three or four organs are affected. The disease occurs predominantly in older men and responds well to steroid therapy. Serum IgG4 levels and immunos-taining with anti-IgG4 antibody are useful in making the diagnosis. Since malignant tumors are frequently suspected on initial presentation, IgG4-related sclerosing disease should be considered in the differential diagnosis to avoid unnecessary surgery.
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.
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.
