Evaluation of two functional CD24 polymorphisms in primary sclerosing cholangitis

Abstract

Background: Primary sclerosing cholangitis (PSC) is a progressive liver disease and characterized by chronic inflammation, sclerosis and strictures of bile ducts. Several genetic risk factors might contribute to pathogenesis. Functional single nucleotide polymorphisms (SNPs) in the CD24 gene have been associated with the development of autoimmune and autoinflammatory diseases and might contribute to the susceptibility for inflammatory bowel disease (IBD).

Aim: This retrospective study aimed to evaluate the impact of two functional CD24 SNPs on clinical features and disease progression in patients with PSC.

Methods: A C to T coding polymorphism (rs8734) and a TG deletion in the 3´- untranslated region (rs3838646) were genotyped. The study cohort comprises of 359 PSC patients for rs3838646 genotype and 335 PSC patients for rs8734 genotype. Clinical and laboratory parameters were collected by chart review.

Results: For the rs8734 genotype, 175 patients (52.2%) were found to be homozygous wildtype (‘Ala/Ala’), 127 (37.9%) patients were heterozygous (‘Ala/Val’) and 33 patients (9.9%) were homozygous mutant (‘Val/Val’). The rs8734genotype was associated with a decreased risk for dominant strictures at first diagnosis of PSC (p?=?.04). For the rs3838646 genotype, 322 patients (89.7%) were found to be homozygous wildtype (‘TG/TG’); 37 showed the ‘TG/del’ genotype (10.3%). The ‘TG/del’genotype was associated with alower risk of IBD (p?=?.01).There was no influence of both CD24 SNPs with clinical end points or transplantation-free survival in our PSC cohort.

Conclusion: Our results suggest a mild association of the rs8734 CD24 genotype with dominant strictures at first diagnosis of PSC. The rs3838646 CD24 genotype is associated with a lower rate of IBD. Both SNPs seem to modulate the clinical phenotype without major pathogenetic importance for disease progression in PSC.     

Mortality and extraintestinal cancers in patients with primary sclerosing cholangitis and inflammatory bowel disease

IntroductionPrimary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD) frequently co-occur. PSC is associated with increased risk for colorectal cancer (CRC). However, whether PSC is associated with increased risk of extraintestinal cancers or affects mortality in an IBD cohort has not been examined previously.MethodsIn a multi-institutional IBD cohort of IBD, we established a diagnosis of PSC using a novel algorithm incorporating narrative and codified data with high positive and negative predictive value. Our primary outcome was occurrence of extraintestinal and digestive tract cancers. Mortality was determined through monthly linkage to the social security master death index.ResultsIn our cohort of 5506 patients with CD and 5522 patients with UC, a diagnosis of PSC was established in 224 patients (2%). Patients with IBD–PSC were younger and more likely to be male compared to IBD patients without PSC; three-quarters had UC. IBD–PSC patients had significantly increased overall risk of cancers compared to patients without PSC (OR 4.36, 95% CI 2.99–6.37). Analysis of specific cancer types revealed that a statistically significant excess risk for digestive tract cancer (OR 10.40, 95% CI 6.86–15.76), pancreatic cancer (OR 11.22, 95% CI 4.11–30.62), colorectal cancer (OR 5.00, 95% CI 2.80–8.95), and cholangiocarcinoma (OR 55.31, 95% CI 22.20–137.80) but not for other solid organ or hematologic malignancies.ConclusionsPSC is associated with increased risk of colorectal and pancreatobiliary cancer but not with excess risk of other solid organ cancers.     

Inflammatory bowel disease course in liver transplant versus non-liver transplant patients for primary sclerosing cholangitis: LIVIBD,an IG-IBD study

BackgroundData regarding the effect of orthotopic liver transplantation (OLT) for primary sclerosing cholangitis (PSC) on inflammatory bowel disease (IBD) course are scarce and conflicting.AimsTo compare the incidence of refractory IBD in two groups (OLT and non-OLT) of patients affected by IBD and PSC.MethodsAn observational, multicentre, cohort retrospective study was conducted by the Italian Group for the study of IBD in Italy. The primary outcome was the need for biologic therapy or bowel resection for medically refractory IBD or hospitalization due to IBD relapse during the follow-up. Secondary outcomes were rate of colonic dysplasia, colorectal cancer, other solid tumours, lymphoma.ResultsEighty-four patients were included in the study. The primary outcome was not different between OLT and non-OLT groups (11/27, 40.7%, versus 20/57, 35.1%, respectively, p = 0.62). The lymphoma and other tumours (thyroid cancer, kidney cancer, ileal tumour, ovarian cancer, cervical cancer) rates were significantly higher in the OLT group (p = 0.04 and p = 0.005, respectively), at the limit of statistical significance for high-grade colonic dysplasia (p = 0.06).ConclusionOLT in patients affected by IBD and PSC is not a risk factor for a more severe IBD course, but it is associated with a higher occurrence of cancer.     

Perihilar lymph nodes in patients with primary sclerosing cholangitis with and without cholangiocellular carcinoma

Objective. Enlarged perihilar lymph nodes have been described in patients with primary sclerosing cholangitis (PSC). The aim of the study was to determine the clinical relevance of perihilar lymph nodes in PSC patients with and without cholangiocellular carcinoma (CCC). Material and methods. The status of perihilar lymph nodes was investigated in 117 patients with PSC using “high-end” ultrasound. Thirty-five of the 117 PSC patients had histologically proven CCC. Lymph node status was correlated with the presence of CCC and inflammatory bowel disease (IBD). Results. Seventy-three percent of PSC patients without CCC and 86% of patients with CCC had enlarged perihilar lymph nodes (NS). In CCC patients, the width of lymph nodes was significantly larger (12±6 mm versus 8±4 mm; p=0.0001), and the length:width ratio (2.15±0.7:1 versus 2.5±0.6:1; p=0.004) of the lymph nodes was significantly lower. Thirty-seven percent of PSC patients without CCC and 57% of patients with PSC and CCC had multiple perihilar lymph nodes (p=0.04). In all patients, the presence versus absence of IBD had no influence on the number (84% versus 74%,) and size of perihilar lymph nodes (length: 21±10 mm versus 19±7 mm). Lymph node status did not correlate with the number of episodes of cholangitis. Conclusions. Enlarged perihilar lymph nodes are characteristic of patients with PSC. Since perihilar lymph nodes are not predictive of the presence of complicating CCC, such patients should not be excluded from liver transplantation.     

Neoplasia in the ileoanal pouch following colectomy in patients with ulcerative colitis and primary sclerosing cholangitis

Background & AimsPrimary sclerosing cholangitis (PSC) is typically associated with inflammatory bowel disease (IBD), particularly ulcerative colitis (UC). PSC–IBD patients are at an increased risk for colorectal neoplasia. The ileal pouch-anal anastomosis (IPAA) is a treatment option for patients with medically refractory UC or neoplasia. However, little is known about the development of pouch neoplasia in PSC–UC patients following an IPAA. We aim to describe the incidence of pouch neoplasia in PSC–UC patients after an IPAA.MethodsWe conducted a retrospective chart review of patients with a confirmed diagnosis of PSC and IBD who underwent colectomy with IPAA followed by pouch surveillance between 1995 and 2012.ResultsSixty-five patients were included in the cohort and were followed up from the time of colectomy/IPAA for a median of 6 years. The most common indications for surgery were low-grade dysplasia (LGD) and refractory colitis. Only 3 patients developed evidence of neoplasia (LGD n = 1, high-grade dysplasia n = 1, adenocarcinoma n = 1). The cumulative 5-year incidence of pouch neoplasia was 5.6% (95% confidence intervals [CI], 1.8%–16.1%).ConclusionBased on our short-term follow-up, surveying the pouch frequently appears to be an unnecessary practice in PSC–IBD patients. Longer follow-up will be needed to develop an optimal surveillance strategy for the development of dysplasia and cancer in such patients.     

Risk of inflammatory bowel disease after Campylobacter jejuni and Campylobacter concisus infection: a population-based cohort study

Objectives: In this population-based cohort study, we aimed to examine the risk of IBD following a positive stool culture with Campylobacter jejuni or Campylobacter concisus, as well as following culture-negative stool testing.

Materials and methods: Patients with a first-time positive stool culture with C. jejuni or C. concisus, as well as negative stool testing, from 2009 through 2013 in North Denmark Region, Denmark, were identified. Patients diagnosed with IBD during follow-up (to 1 March 2018) were identified using national registries. For each case, we selected ten population comparisons matched by age, gender, and calendar-time.

Results: We identified 1693 patients with C. jejuni, 910 C. concisus-positive patients, and 11,383 patients with culture-negative stools. During the first year of follow-up C. jejuni-positive patients had higher risk of IBD (HR 2.2, 95% CI 1.3–3.7) compared to population comparisons, but not after exclusion of the first year (HR 1.1, 95% CI 0.5–2.3). Campylobacter concisus-positive patients and culture-negative patients had similar risk of IBD (HR 12.9, 95% CI 7.2–22.9 and HR 8.7, 95% CI 7.5–10.2), during the first year, which decreased to (HR 3.3, 95% CI 1.3–8.5 and HR 3.2, 95% CI 2.6–4.0) after exclusion of the first year.

Conclusions: This study does not support exposure of C. jejuni or C. concisus infection as a causal trigger in subsequent development of IBD, since culture-negative patients had similar risk for IBD on long term follow-up. Additional studies including C. concisus exposures for an evaluation of the specific risk of IBD are needed.     

Fatigue in patients with primary sclerosing cholangitis

Background: The occurrence of fatigue in primary sclerosing cholangitis (PSC), its impact on quality of life and the role of concomitant inflammatory bowel disease (IBD) and coexisting irritable bowel syndrome (IBS) is unexplored. Methods: Ninety‐three patients with PSC, associated with IBD in 80% of cases and 77 patients with IBD alone, were enrolled in the study. The patients completed the following questionnaires: the Fatigue Impact Scale (FIS), the Psychological General Well‐Being Index (PGWB), the Gastrointestinal Symptom Rating Scale (GSRS), the Beck Depression Inventory (BDI) and diagnostic criteria for IBS. Questionnaire data were related to liver tests and the latest liver biopsy in the PSC patients. Two sex‐ and age matched controls from the general population (GP) were assigned to each PSC patient and these controls completed the FIS and the BDI. Results: Total fatigue score did not differ significantly between patients with PSC and IBD alone. Median total fatigue score among GP subjects was 39 (13–72), which was higher than in PSC (19 (6–52) (P?=?0.02)) and in IBD patients (19 (5–35) (P?Conclusions: Fatigue in patients with PSC is related to depression but not to the severity of the liver disease. Both the PSC and IBD patients had lower total fatigue scores than subjects from the general population. This argues against fatigue as a specific symptom of PSC and IBD patients.     

Colitis-associated sclerosing cholangitis in children: A single centre experience

ObjectiveSclerosing cholangitis (SC) is an important immune-mediated extra-intestinal manifestation of inflammatory bowel disease (IBD), primarily affecting patients with ulcerative colitis (UC). The reported prevalence of SC in adults and children with UC is low at between 2 and 7%. We present findings from a hepatological work-up in children with inflammatory colitis and elevated liver function tests (LFT) from a tertiary paediatric gastroenterology unit.DesignThis study is designed as a retrospective review of the medical records of 17 children and adolescents with inflammatory colitis and abnormal LFTs who presented to our IBD service between April 2004 and April 2012.ResultsOver the eight year period a total of 52 patients were diagnosed with inflammatory colitis (ulcerative colitis and unclassified colitis). Seventeen of the 52 patients had abnormal liver function tests and underwent liver biopsy and cholangiography. All 17 patients (32.6%) were diagnosed with hepato-biliary disease.ConclusionThis is one of the largest reported series of children with inflammatory colitis and associated hepato-biliary disease. The data from this patient group indicate that the prevalence of IBD-associated hepato-biliary disease in children with abnormal LFTs is much higher than previously reported. As the diagnosis of IBD-associated hepato-biliary disease affects patient management, we recommend liver biopsy and cholangiography in all children with inflammatory colitis and abnormal liver function tests.     

Inflammatory bowel disease after liver transplantation: A role for cytomegalovirus infection

Objective. Despite the use of immunosuppressive drugs, recurrent and de novo inflammatory bowel disease (IBD) can develop after orthotopic liver transplantation (OLT). Cytomegalovirus (CMV) infection has been suggested to play a role in the pathogenesis of IBD. The aim of this study was to investigate the role of CMV infection in the development of IBD after OLT. Material and methods. All 84 patients who underwent transplantation for primary sclerosing cholangitis (PSC) or autoimmune hepatitis (AIH) in our center between May 1987 and June 2002 and who survived the first year after transplantation were included in the study. Diagnosis of active CMV infection was made using the pp65–antigenemia assay. Results. Thirty-one of the 84 patients (37%) had IBD prior to OLT. Eighteen patients (21%) experienced IBD after OLT, either as flare-up (n=12) or de novo (n=6), at a median of 1.4 years (range 0.3 to 6.3) after OLT. Forty-eight percent of all patients experienced CMV infection after OLT, at a median of 27 days (range 8 to 193). CMV infection was primary in half the patients. At 1, 3, and 5 years after OLT, active IBD-free survival without CMV infection was 91, 88, and 88%, respectively. With CMV infection these figures were 93, 82, and 67%. De novo IBD was seen only in those who had experienced a CMV infection (p=0.02). Conclusions. In patients transplanted for end-stage PSC or AIH, active IBD, especially de novo IBD, occurred more often in patients who experienced CMV infection in the postoperative period. This finding supports a pathogenic role for CMV in the development of IBD.     

Clinical aspects and prognosis of patients with inflammatory bowel disease associated with autoimmune liver diseases

Background and aimsInflammatory bowel diseases (IBD) are chronic conditions that may be accompanied by autoimmune liver disease (AILD), most commonly primary sclerosing cholangitis (PSC). The objective of this study was to evaluate the behaviour of patients with IBD associated with AILD and compare a PSC group with a non-PSC group.MethodsMedical records of patients with IBD associated with PSC, autoimmune cholangitis, primary biliary cholangitis, small-duct PSC, autoimmune hepatitis (AIH) and overlapping syndromes were assessed.ResultsFifty-four patients were included: 48 (88.9%) had ulcerative colitis and six (11.1%) had Crohn's disease; 35 (64.8%) had PSC and 19 (35.2%) did not have PSC. There was no difference in outcomes (surgical treatment for IBD, liver transplantation or death) between the groups. Time since the diagnosis of IBD was associated with surgical treatment of IBD (p = 0.041; OR: 1.139, 95% CI: 1.006–1.255). Time since the diagnosis of AILD (p = 0.003; OR: 1.259, 95% CI: 1.1–1.396), as well as portal hypertension at diagnosis (p = 0.014; OR: 18.22, 95% CI: 1.815–182.96), were associated with liver transplantation. In addition, previous diagnosis of AIH was associated with de novo IBD (p = 0.012; OR: 7.1, 95% CI: 1.215–42.43).ConclusionBoth groups had similar disease behaviour. A longer time since the diagnosis of IBD increased the risk for surgical treatment (13.9%/year). A 25.9%/year increase in liver transplantation was observed after the diagnosis of AILD, which was increased 18.22 times by the presence of portal hypertension. In addition, the diagnosis of AIH was associated with an increase in the number of diagnoses of de novo IBD (7.1).     

Subclinical time span of inflammatory bowel disease in patients with primary sclerosing cholangitis

PURPOSE: This study is designed to describe colonic histology in patients with primary sclerosing cholangitis (PSC) without clinical symptoms of inflammatory bowel disease (IBD) and to do a follow-up study of these patients to find the time span from first detection of histologic signs until development of clinical symptoms of IBD. METHODS: In a cohort of 76 patients with PSC treated at Huddinge University Hospital, 11 patients did not have any clinical symptoms of IBD at the time of PSC diagnosis. Nine of these patients underwent diagnostic colonoscopy with multiple biopsies. RESULTS: In the group of nine PSC patients, without clinical signs of IBD undergoing colonoscopy, histologic signs of IBD were found in seven patients (6 ulcerative colitis and 1 Crohn's disease). Among them one had dysplasia, and another had epithelial changes probably positive for dysplasia. Two other patients had histologic signs of inflammation, however, not fully compatible with IBD. Three of 11 patients developed clinical symptoms of IBD after one, three, and seven years of follow-up since diagnostic colonoscopy. CONCLUSIONS: In patients with PSC, histologic signs of IBD, including premalignant changes, may precede development of clinical symptoms of IBD by as much as seven years. This indicates that IBD onset may have a substantial subclinical phase of IBD far longer than previously appreciated. This finding may be of clinical importance because underestimation of disease duration may delay inclusion of PSC patients with extensive colitis in colonoscopic surveillance programs. The subclinical phase may also allow the studies of early pathogenesis in vivo.Supported by grants from the Nanna Svartz Scholarship.     

Caracterización y prevalencia de manifestaciones extraintestinales en una cohorte de pacientes con enfermedad inflamatoria intestinal en Medellín (Colombia)

IntroductionExtraintestinal manifestations (EIMs) are frequent in patients with inflammatory bowel disease (IBD). Our objective is to characterize and determine the prevalence of MEIs in our cohort of patients with IBD.Patients and methodsA retrospective study was carried out in adult patients with IBD at the Pablo Tobón Uribe Hospital in Medellín. Colombia. Articular MEIs, primary sclerosing cholangitis (PSC), both ophthalmological and dermatological, were considered. Absolute and relative frequencies were used. The Chi square test of independence was used to compare 2 proportions and the odds ratio (OR) was estimated.ResultsOur registry has 759 patients with IBD, 544 present UC (71.6%), 200 CD (26.3%) and 15 unclassifiable IBD (1.9%); 177 patients with IBD (23.3%) presented EIMs, 123 of 544 (22.6%) with UC and 53 of 200 (26.5%) with CD (OR: 0.81, 95% CI: 0.55-1.17, P = 0.31). Regarding the type of EIMs, the articular ones were the most frequent (13.5%), more in CD than in UC (20.0 vs. 11.3%, OR 1.94, 95% CI: 1.25-3.00, P = 0.0037). Patients with IBD and EIMs used more antibodies against tumor necrosis factor (anti-TNFs), compared to those without EIMs (43.5 vs. 18.5%, OR 3.38, 95% CI: 2.31-4.90, P = 0.0001).ConclusionsThe prevalence of EIMs in our cohort is high (23.3%) and the most frequent type is joint. Anti-TNFs are most used when IBD and EIMs coexist. Our study provides valuable information on the association of EIMs and IBD in Latin America.     

Comparing guideline-based care quality for inflammatory bowel disease and rheumatoid arthritis patients within a medical home

Background: Rheumatoid arthritis (RA) and inflammatory bowel disease (IBD) patient populations face similar risks of chronic immunosuppression including corticosteroid use. We compared the receipt of preventive services between IBD and RA populations according to published quality metrics.

Methods: We defined a single-center cohort of patients with IBD or RA receiving specialty and primary care. Electronic health record abstraction assessed quality metrics, sociodemographics, comorbidity, and utilization. Comparisons used multivariate odds ratios and Student’s t-tests.

Results: 218 RA and 190 IBD patients were included. In multivariate analysis, IBD patients were less likely to receive pneumococcal vaccination (OR=0.29, 95% CI: 0.11-0.85), while RA patients underwent glucocorticoid-induced osteoporosis screening more often (100% vs. 82.5%, p = 0.023).

Conclusions: Gastroenterologists can improve care quality for IBD patients by assuming greater responsibility for preventive care in IBD patients and/or collaborating with primary care and health systems to improve preventive care delivery.     

Colorectal neoplasia in patients with primary sclerosing cholangitis undergoing liver transplantation: a Nordic multicenter study

Abstract

Objective. Several studies have implicated primary sclerosing cholangitis (PSC) as an additional risk factor for colorectal neoplasia in inflammatory bowel disease (IBD). Some reports have indicated that the risk is even higher in PSC-IBD patients after liver transplantation (Ltx), but this issue is controversial. We aimed to compare the risk of colorectal neoplasia in PSC-IBD patients before and after Ltx and to identify risk factors for colorectal neoplasia post-transplant. Material and methods. In a multicenter study within the Nordic Liver Transplant Group, we assessed the risk of colorectal neoplasia by using the competing risk regression analysis. Results. Among the 439 PSC patients included, 353 (80%) had IBD at the time of Ltx and 15 (3%) patients developed de novo IBD post-Ltx. The median duration of IBD was 15 (0–50) years at the time of Ltx and follow-up after Ltx was 5 (0–20) years. Ninety-one (25%) PSC-IBD patients developed colorectal neoplasia. The cumulative risk of colorectal neoplasia was higher after than before Ltx (HR: 1.9, 95% CI: 1.3–2.9, p = 0.002). A multivariate analysis demonstrated aminosalicylates and ursodeoxycholic acid as significantly associated with an increased risk of colorectal neoplasia post-Ltx. Duration and activity of IBD did not significantly affect the risk of neoplasia. Conclusion. The even higher risk of colorectal neoplasia in PSC-IBD patients after when compared with that of before Ltx underscores the importance of regular surveillance colonoscopies post-Ltx. The association of aminosalicylates and ursodeoxycholic acid to the development of colorectal neoplasia after Ltx should be further investigated.     

Interim analysis of a multicenter registry study of COVID-19 patients with inflammatory bowel disease in Japan (J-COSMOS)

Background

The spread of coronavirus disease 2019 (COVID-19) had a major impact on the health of people worldwide. The clinical background and clinical course of inflammatory bowel disease (IBD) among Japanese patients with COVID-19 remains unclear.

Methods

This study is an observational cohort of Japanese IBD patients diagnosed with COVID-19. Data on age, sex, IBD (classification, treatment, and activity), COVID-19 symptoms and severity, and treatment of COVID-19 were analyzed.

Results

From 72 participating facilities in Japan, 187 patients were registered from June 2020 to October 2021. The estimated incidence of COVID19 in Japanese IBD patients was 0.61%. The majority of IBD patients with COVID-19 (73%) were in clinical remission. According to the WHO classification regarding COVID-19 severity, 93% (172/184) of IBD patients had non-severe episodes, while 7% (12/184) were severe cases including serious conditions. 90.9% (165/187) of IBD patients with COVID-19 had no change in IBD disease activity. A logistic regression analysis stepwise method revealed that older age, higher body mass index (BMI), and steroid use were independent risk factors for COVID-19 severity. Six of nine patients who had COVID-19 after vaccination were receiving anti-tumor necrosis factor (TNF)-α antibodies.

Conclusion

Age, BMI and steroid use were associated with COVID-19 severity in Japanese IBD patients.

     

A unique clinical phenotype of primary sclerosing cholangitis associated with Crohn's disease

Background and aimsA distinct clinical phenotype has been demonstrated for ulcerative colitis with concomitant primary sclerosing cholangitis (PSC). The course and behaviour of Crohn's disease (CD) with PSC has, in contrast, never been defined. We aimed to define the characteristics of patients with concomitant PSC and CD.MethodsThe Oxford PSC and IBD databases were abstracted for: PSC subtype, date of diagnosis, symptom onset, smoking history, Mayo Clinic PSC score and outcomes (hepatic failure, liver transplantation, Montréal CD classification, treatment, cancer and death). Patients with PSC/CD were matched 1:2 to two control groups: one with PSC/UC and one with isolated CD.Results240 patients with PSC were identified; 32 (13%) with CD, 129 (54%) with co-existing UC, and 79 had PSC without IBD. For PSC/CD vs. CD controls, isolated ileal CD was less common (6% vs. 31%, p = 0.03). Smoking was less common in PSC/CD (13% vs. 34%, p = 0.045). No difference in the distribution of CD, or treatment required was observed. For PSC/CD vs. PSC/UC controls, more patients with PSC/CD were female (50% vs. 28%, p = 0.021). 22% of PSC/CD patients had small duct PSC compared with 6% with PSC/UC, (p = 0.038). Major event-free survival was prolonged in the PSC/CD group compared with PSC/UC, (Cox regression p = 0.04).ConclusionUnlike PSC/UC, patients with PSC/CD were as likely to be female as male, more commonly had small duct PSC and less commonly progressed to cancer, liver transplantation, or death. Compared to patients with isolated CD, patients with PSC/CD were less likely to smoke or have ileal disease.     

原发性硬化性胆管炎患者炎症性肠病和胆囊息肉发生率分析*

目的 对原发性硬化性胆管炎(PSC)患者炎症性肠病(IBD)和胆囊息肉(GP)发生情况进行回顾性分析。方法 2000年1月～2018年12月我院收治的201例PSC患者,经内镜逆行胰胆管造影(ERCP)和磁共振胰胆管造影(MRCP)诊断,经腹部CT或腹部超声或肠镜检查证实GP和IBD存在。结果 在201例PSC患者中检出GP 23例(11.4%)和IBD 154例(76.6%);在23例GP患者中,IBD、溃疡性结肠炎(UC)和克罗恩病(CD)发生率分别为87.0%、73.9%和8.7%,在178例无GP患者中,则分别为75.3%、58.4%和15.7%,无显著统计学差异(P>0.05);15例GP患者接受了胆囊切除术,术后组织病理学检查提示5例(33.3%)为腺癌,3例(20.0%)为高度不典型增生,另7例为良性病变,良性与恶性病变患者临床资料比较无统计学差异(P>0.05)。结论 PSC患者GP和IBD发病率很高,其中部分GP可进展为恶性病变,宜早期手术治疗。     

Interactions between primary sclerosing cholangitis and inflammatory bowel disease: implications in the adult liver transplant setting

Introduction: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease which is associated with inflammatory bowel disease (IBD) in most cases. As there is currently no medical therapy which alters the natural history of PSC, liver transplantation may be required.

Areas covered: We searched for articles in PubMed and critically reviewed current literature on the interrelationship between PSC and IBD with a specific focus on considerations for patients in the liver transplant setting.

Expert commentary: PSC is an uncommon disease which limits available studies to be either retrospective or contain relatively small numbers of patients. Based on observations from these studies, the behavior and complications of PSC and IBD impact on each other both before and after a liver transplant. Both these autoimmune conditions and their associated cancer risk also influence patient selection for transplantation and may be impacted by immunosuppression use post-transplant. Hence, a complex interplay exists between PSC, IBD and liver transplantation which requires clarification with ongoing research.