A 60-Year-Old Woman with Primary Biliary Cholangitis and Crohn’s Ileitis Following the Suspension of Ursodeoxycholic Acid (original) (raw)
Related papers
Hepatology, 2007
A longitudinal, cohort study was performed to characterize the clinical features of patients with small-duct primary sclerosing cholangitis (PSC) occurring with and without inflammatory bowel disease (IBD) and to determine the influence of IBD and the effect of ursodeoxycholic acid (UDCA) therapy on the course of the liver disease. Forty-two patients with small-duct PSC (14 women and 28 men; mean age, 36.7 ؎ 13.3 years) were followed for up to 24.9 years. At presentation, prevalence of signs of liver disease (none versus 35%, P ؍ 0.002), gastroesophageal varices (5% versus 30%, P ؍ 0.03), and stage III/IV disease (9% versus 45%, P ؍ 0.008) were lower in those with IBD versus those without IBD. During follow-up, 6 patients underwent liver transplantation, and another died of cirrhosis. Using the Cox proportional hazard analysis, concomitant IBD was not associated with liver death or transplant, whereas the revised Mayo risk score for PSC was the only prognostic factor associated with liver-related outcomes (relative risk, 6.47; 95% confidence interval, 1.75-137.5). UDCA (13-15 mg/kg/day) therapy for an average of 40 months showed biochemical improvement (P < 0.001) in UDCA-treated patients, while no significant change occurred in untreated patients. UDCA therapy had no effect on delaying progression of disease (relative risk, 0.95; 95% confidence interval, 0.38-2.36). Conclusion: Small-duct PSC often is recognized at an early stage in patients with IBD; however, IBD has no impact on long-term prognosis. Although UDCA therapy improves liver biochemistries, it may not delay disease progression during the short period of treatment. (HEPATOLOGY 2008;47: 133-142.) S mall-duct primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown etiology characterized by fibrosing inflammation and destruction of interlobular and septal bile ducts in the absence of cholangiographic evidence of sclerosing cholangitis. 1,2 Small-duct PSC represents approximately 6% to 11% of patients with sclerosing cholangitis and commonly coexists with inflammatory bowel disease (IBD). 3,4 Three groups have reported follow-up information from patients with small-duct PSC, which showed that the clinical course of small-duct PSC was more benign than large-duct PSC. 3-5 Unfortunately, some patients with small-duct PSC follow a progressive course over time, develop the typical cholangiographic features of large-duct PSC, and advance to biliary cirrhosis and its complications with the consequent necessity of liver transplantation. 3-5 However, given that the definition of small-duct PSC varied from study to study in terms of evidence of IBD, the influence of concomitant IBD on the long-term prognosis of patients with small-duct PSC remains largely unstudied. Ursodeoxycholic acid (UDCA) is a hydrophilic bile acid that is widely used in the treatment of cholestatic liver disease of all causes. UDCA appears to exert a number of effects, all of which may be beneficial in chronic cholestasis: a choleretic effect by an increase in bile flow, a direct cytoprotective effect, an indirect cytoprotective effect by displacement of the more hepatotoxic endogenous hydro
2021
Introduction: Primary biliary cholangitis (PBC), the new dominance of primary biliary cirrhosis, is a cholestatic disease of autoimmune etiology and represents the leading cause of intra-hepatic cholestasis. Treatment is mainly based on ursodeoxycholic acid. The biological response to treatment is the main predictor of survival without liver transplantation. The Globe-score has been recently validated as the main prognostic factor. Materials and methods: This is a retrospective study carried out in our department collating all cases of PBC followed in consultation. The aim of our work is to research the predictors of poor response to UDCA. Results: 46 patients were collected. The mean age of the patients was 58.82 years, with a predominance of women (n = 43, 93.5%). 34.78% of patients were in the stage of cirrhosis. Anti-M2 mitochondria antibodies were positive in 44 patients (95.65%). An overlap syndrome was found in 11 patients (23.9%). Treatment was based on UDCA combined with co...
Autoimmune Diseases Associated with Primary Biliary Cholangitis
Saudi Journal of Medical and Pharmaceutical Sciences, 2019
Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease; the autoimmune mechanism seems the most likely. As a result, PBC is frequently associated with other autoimmune diseases. The goal of our work is to determine the prevalence and type of autoimmune diseases associated with PBC and to assess their impact on the prognosis of the disease. Materials and methods: This is a retrospective study over a period of 22 years (1998-2019) including all patients followed for CBP. In all these patients, we systematically looked for: autoimmune hepatitis, dysthyroidism, and type 1 diabetes, dry syndrome, and celiac disease, dermatological and joint damage. The statistical analysis of the data was done using the SPSS software. The comparison of the biochemical response to ursodeoxycholic acid between patients with isolated CBP and those with CBP associated with autoimmune pathology was performed using the Chi2 test. A combination of variables was considered statistically significant if p < 0.05. Results: 90 patients (85 women and 5 men) followed for CBP were collected. The average age was 49+/-12.3 years. Of these patients, 36 patients (42.9%) had an autoimmune disease associated with PBC. The discovery of these autoimmune diseases preceded the diagnosis of PBC in 9 cases (27.3%) and was concomitant in the remaining cases. Autoimmune hepatitis was found in 10 patients (12%), defining an overlapping syndrome. Other diseases were Hashimoto's thyroiditis (n = 9), basedow (n=1) dry syndrome (n = 10), celiac disease (n = 3), insulin-dependent diabetes (n = 2), systemic scleroderma (n = 1), rheumatoid arthritis (n = 1), Addisson disease (n=1) Psoriasis (n = 1) vitiligo (n = 1). The comparison of the biochemical response to ursodeoxycholic acid between patients with isolated CBP and those with CBP associated with autoimmune pathology was statistically non-significant with p=0.67. Conclusion: In our series, the prevalence of autoimmune diseases associated with PBC was 40%. These diseases were dominated by autoimmune hepatitis, Hashimoto's thyroiditis and dry syndrome. Although their association does not appear to alter the prognosis for CBP or the response to AUDC, their screening must be systematic in order to initiate early and appropriate treatment. Although ursodeoxycholic acid is beneficial in the treatment of primary biliary cirrhosis, it has had no measurable effect on the autoimmune disorders associated with the disease.
Inflammatory Bowel Diseases, 2013
Background: Ursodeoxycholic acid (UDCA) may modify the risk of inflammatory bowel disease (IBD)-associated colorectal cancer. We performed a systematic review and meta-analysis of studies evaluating the effect of UDCA on the risk of IBD-associated colorectal neoplasia (CRN) (defined as colorectal cancer and/or dysplasia) in patients with primary sclerosing cholangitis with concomitant IBD (PSC-IBD). Methods: We conducted a systematic search of Medline, Embase, and Web of Science and manually reviewed the literature. Studies were included if they: (1) evaluated exposure to UDCA in patients with PSC-IBD, (2) reported IBD-associated CRN as outcome, and (3) reported relative risks or odds ratios (ORs) or provided data for their calculation. Summary OR estimates with 95% confidence intervals (CIs) were calculated using the random-effects model. Results: Eight studies (5 observational, 3 randomized controlled trials) reporting 177 cases of CRN in 763 patients with PSC-IBD were included in the analysis. Overall, meta-analysis showed no significant protective association between UDCA use and CRN (OR, 0.81; 95% CI, 0.41-1.61). However, there was a significant chemopreventive effect on the risk of advanced CRN (colorectal cancer and/or high-grade dysplasia) (OR, 0.35; 95% CI, 0.17-0.73). In a subgroup analysis, low-dose UDCA use (8-15 mg/kg/d) was associated with significant risk reduction of CRN (OR, 0.19; 95% CI, 0.08-0.49). Conclusions: UDCA, particularly at low doses, may reduce the risk of advanced CRN in patients with PSC-IBD. However, results should be interpreted with caution, given limited reporting of cancer-related outcomes, primarily from tertiary care centers.
Journal of Medical and Surgical Research, 2021
Introduction: Primary biliary cholangitis (PBC), the new dominance of primary biliary cirrhosis, is a cholestatic disease of autoimmune etiology and represents the leading cause of intra-hepatic cholestasis. Treatment is mainly based on ursodeoxycholic acid. The biological response to treatment is the main predictor of survival without liver transplantation. The Globe-score has been recently validated as the main prognostic factor. Materials and methods: This is a retrospective study carried out in our department collating all cases of PBC followed in consultation. The aim of our work is to research the predictors of poor response to UDCA. Results: 46 patients were collected. The mean age of the patients was 58.82 years, with a predominance of women (n = 43, 93.5%). 34.78% of patients were in the stage of cirrhosis. Anti-M2 mitochondria antibodies were positive in 44 patients (95.65%). An overlap syndrome was found in 11 patients (23.9%). Treatment was based on UDCA combined with co...
Hepatopancreatobiliary manifestations of inflammatory bowel disease
Clinical Journal of Gastroenterology, 2012
Inflammatory bowel disease (IBD) is frequently associated with extraintestinal manifestations such as hepatopancreatobiliary manifestations (HPBMs), which include primary sclerosing cholangitis (PSC), pancreatitis, and cholelithiasis. PSC is correlated with IBD, particularly ulcerative colitis (UC); 70-80% of PSC patients in Western countries and 20-30% in Japan have comorbid UC. Therefore, patients diagnosed with PSC should be screened for UC by total colonoscopy. While symptoms of PSC-associated UC are usually milder than PSC-negative UC, these patients have a higher risk of colorectal cancer, particularly in the proximal colon. Therefore, regular colonoscopy surveillance is required regardless of UC symptoms. Administration of 5-aminosalicylic acid or ursodeoxycholic acid may prevent colorectal cancer and cholangiocarcinoma. While PSC is diagnosed by diffuse multifocal strictures on cholangiography, it must be carefully differentiated from immunoglobulin G4 (IgG4)-associated cholangitis, which shows a similar cholangiogram but requires different treatment. When PSC is suspected despite a normal cholangiogram, the patient may have small-duct PSC, which requires a liver biopsy. IBD patients have a high incidence of acute and chronic pancreatitis. Most cases are induced by cholelithiasis or medication, although some patients may have autoimmune pancreatitis (AIP), most commonly type 2 without elevation of serum IgG4. AIP should be accurately identified based on characteristic image findings, because AIP responds well to corticosteroids. Crohn's disease is frequently associated with gallstones, and several risk factors are indicated. HPBMs may influence the management of IBD, therefore, accurate diagnosis and an appropriate therapeutic strategy are important, as treatment depends upon the type of HPBM.
The Indonesian Journal of Gastroenterology, Hepatology, and Digestive Endoscopy
Primary biliary cirrhosis (PBC) is an autoimmune liver disease of unknown etiology and is characterized by chronic progressive cholestasis with destruction of the small intrahepatic bile ducts and associated most commonly with antimitochondrial antibodies. PBC is most common in women and is often associated with other autoimmune disease such as autoimmune hemolityc anemia (AIHA), rheumatoid arthritis, thyroiditis, and systemic lupus eritomatosus. We report one case, a 20 years old woman with AIHA have been treated by corticosteroid since last year and she came to the outpatient department (OPD) with fatique and jaundice. The result of laboratory were haemoglobin 8.7 mg/dL, white blood cell 8700 mg/dL, coomb test +2, total bilirubin 33.2 mg/dL, direct bilirubin 29.3 mg/dL, γGT: 297 mg/dL and alkalyphospatase: 158 mg/dL. The result of Abdominal CT scan showed the size of liver and spleen increased and normal common bile duct (CBD). The result of ANA test, anti-nuclear (ANA) and antimitochondrial M2 (AMA M2) antibodies were positive. From the physical examination, laboratory and CT scan Abdomen; the diagnose of this patient was AIHA with PBC. After treatment with corticosteroid (prednison 1mg/kg/day) and ursodeoxicholic acid (UDCA) for several weeks, the clinical manifestation of PBC such as jaundice getting better (the laboratory result: total bilirubin 2.7 mg/dL, direct bilirubin 1.5 mg/dL, gamma GT 80 mg/dL).
High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis
Hepatology, 2009
Background-Previous controlled trials are inconclusive regarding the efficacy of ursodeoxycholic acid (UDCA) for treating primary sclerosing cholangitis (PSC). Methods-One hundred fifty adult patients with PSC were enrolled in a long-term, randomized, double-blind controlled trial of high-dose UDCA (28-30 mg/kg/day) versus placebo. Liver biopsy and cholangiography were performed before randomization and after 5 years. The primary outcome measure was development of cirrhosis, varices, cholangiocarcinoma, liver transplantation or death. Results-The study was terminated after six years because of futility. At enrollment, the UDCA (n=76) and placebo (n=74) groups were similar with respect to gender, age, duration of disease, serum aspartate aminotransferase (AST) and alkaline phosphatase (AP) levels, liver histology and Mayo risk score. During therapy, AST and AP levels decreased more in the UDCA than the placebo group (p<0.01) but improvements in liver tests were not associated with decreased endpoints. By the end of the study, 30 patients in the UDCA group (39%) versus 19 patients in the placebo group (26%) had reached one of the pre-established clinical endpoints. After adjustment for baseline stratification characteristics, the risk of a primary endpoint was 2.3 times greater for patients on UDCA than for those on placebo (p<0.01) and 2.1 times greater for death, transplantation, or minimal listing criteria (p=0.038). Serious adverse events were more common in the UDCA than placebo group (63% vs 37%: p<0.01). Conclusions-Long-term high-dose UDCA therapy is associated with improvement in serum liver tests in PSC but does not improve survival and was associated with higher rates of serious adverse events.