Pregnancy in primary sclerosing cholangitis (PSC) is not well studied. There is little known regarding how pregnancy might affect PSC, and how the disease might affect the fetus. In general, advice regarding conception for a woman with PSC reflects knowledge gleaned from a few small studies and case reports 1–5 A common question for a newly diagnosed patient with PSC pertains to future fertility and childbearing. The case series published in this issue of Gut by Wellge and colleagues6 ( see page 1117 ) provides an important look at pregnancy in PSC. In the review by Wellge et al , 229 patients with PSC were evaluated as were 569 healthy controls. The authors found that the number of children was not different between the two groups. This would imply that fertility was not affected by the diagnosis of PSC. A total of 17 patients with known PSC and …
Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease of unknown aetiology. Up to 10% of patients with typical features of PBC will have additional features of autoimmune hepatitis (AIH). A subset, however, have no such features but go on to develop a 'sequential' AIH overlap syndrome.Describe our experience with eight patients who developed AIH after the diagnosis of PBC was made.We reviewed the charts of all PBC patients over a 9-year period (from 1996 to 2005). Only PBC patients with no features of AIH were included.There were 1476 patients with PBC. Of these, eight patients developed features of AIH overlap syndrome based on biochemical and histological parameters. Treatment included prednisone and azathioprine for 24 or more months. The majority of patients remained on ursodeoxycholic acid (UDCA) throughout treatment. Response to therapy was defined by improvement in enzymes, and was rapid for all patients. One patient was able to discontinue treatment with prednisone and azathioprine, while seven have continued on therapy to date.A 'sequential' overlap syndrome of AIH with PBC can occur. Treatment with prednisone and azathioprine may lead to a rapid improvement in aminotransferase levels.
Purpose: To determine if changes in aspartate aminotransferase (AST) values in patients with varying alkaline phosphatase (ALP) status are associated with clinical outcomes in patients with primary sclerosing cholangitis (PSC). Methods: Patients from an earlier study were stratified according to ALP status (normalized ALP vs. persistently elevated ALP) and then their medical records were reviewed for various AST parameters (baseline AST, average AST and highest AST levels during follow-up) and analyzed to determine if an association exists between AST levels, ALP status (normalized vs. persistently elevated) and clinical end points (cholangiocarcinoma, liver transplantation and death). Results: Eighty-seven patients were included in the final analysis. Of the 35 patients with normalized ALP, 18 patients had normal AST (< 48 U/L) at baseline. Similarly, of the 52 patients with persistently elevated ALP, 17 patients had normal AST at baseline (p>0.05). In patients with normalized ALP during follow-up, AST levels were similar among patients regardless of the development of clinical endpoints. Conversely, in the group of patients with elevated ALP, AST levels were significantly higher in the patients developing clinical end-points (average AST=115 U/L, highest AST=535 U/L) as compared to patients not developing clinical endpoints (average AST=81 U/L and highest AST=181 U/L, p=0.03 and p=0.04, respectively) during follow-up. When stratified by usage of ursodeoxycholic acid (UDCA), 25.5 % (12/47) of patients on UDCA developed endpoints as compared to 25% (10/40) not receiving UDCA who developed endpoints (p=0.90). Conclusion: In PSC patients with persistently elevated ALP values, patients developing endpoints were found to have higher AST levels as compared to patients not developing endpoints. Conversely, patients with normalized ALP did not show a similar correlation between serum AST levels and clinical outcomes.
Cirrhosis is a complex disease that is associated with disturbances in different organs besides the liver, including kidneys, heart, arterial circulation, lungs, gut, and brain. As a consequence, patients develop a number of complications that result in frequent hospital admissions and high morbidity and mortality. Patients with cirrhosis require constant and rigorous monitoring both in and outside the hospital. In this context, the role of nurses in the care of patients with cirrhosis has not been sufficiently emphasized and there is very limited information about nursing care of patients with cirrhosis compared with other chronic diseases. The current article provides a review of nursing care for the different complications of patients with cirrhosis. Nurses with specific knowledge on liver diseases should be incorporated into multidisciplinary teams managing patients with cirrhosis, both inpatient and outpatient. Conclusion: Nurses play an important role in the management and prevention of complications of the disease and improvement in patients’ quality of life and bridge the gap between clinicians and families, between primary care and hospital care, and provide medical education to patients and caregivers.
Primary sclerosing cholangitis (PSC) is a chronic, progressive cholestatic biliary disease associated with inflammatory bowel disease (IBD) with no known cure.To evaluate the effect of biological therapies on PSC progression in IBD patients.We performed a retrospective cohort study of 88 cases (75 unique patients with 12 patients treated >1 biologics) of IBD (48 ulcerative colitis, 24 Crohn's disease and 3 indeterminate colitis) with concomitant PSC who received biological therapy (42 infliximab, 19 adalimumab, 27 vedolizumab) between June 2002 and October 2017. Hepatic biochemistries were compared using the paired t-test (patients served as their own controls) ≤3 months before and 6-8 and 12-14 months after biological initiation. Radiographic information of biliary stenosis and liver fibrosis were obtained via abdominal ultrasound, abdominal magnetic resonance imaging and magnetic resonance elastography.Use of adalimumab was associated with a significant decrease in alkaline phosphatase (ALP) after 6-8 months (P = 0.03; mean change -70 U/L, standard deviation [SD] 88 U/L) compared to vedolizumab (mean change +50 U/L, SD 142 U/L) or infliximab (mean change +37 U/L, SD 183 U/L) but the change was not significant after 12-14 months (P = 0.24). No significant decreases were observed with AST, ALT, total or direct bilirubin, elastography score or radiographic imaging of biliary tree dilation/strictures with any biological therapy after 6-8 or 12-14 months.Current evidence suggests that biological therapies used for the treatment of IBD are not effective treatments for PSC. Further study is needed to elucidate any potential beneficial effect of adalimumab on PSC.
Liver stiffness (LS) measured by magnetic resonance elastography (MRE) is emerging as an important biomarker in chronic liver diseases. We examined the diagnostic performance of MRE, factors associated with an increased LS and the prognostic value of LS as measured by MRE among patients with primary sclerosing cholangitis (PSC).We performed a retrospective review of 266 patients with PSC to examine whether LS was associated with the primary endpoint of hepatic decompensation (ascites, variceal hemorrhage and hepatic encephalopathy). The ability of MRE to differentiate stages of fibrosis was examined in a subset of patients who underwent a liver biopsy (n = 20).An LS of 4.93 kPa was the optimal point to detected F4 fibrosis (sensitivity, 1.00; 95% confidence interval (CI), 0.40-1.00; specificity, 0.94; 95%CI, 0.68-1.00). While a serum alkaline phosphatase <1.5 times the upper limit of normal excluded the presence of advanced LS, it was not associated with the primary endpoint (hazard ratio, 0.26; 95%CI, 0.01-1.33). However, LS was associated with the development of decompensated liver disease (hazard ratio, 1.55; 95%CI, 1.41-1.70). The optimal LS thresholds that stratified patients at a low, medium and high risk for hepatic decompensation were <4.5, 4.5-6.0 and >6.0 kPa (respectively).Magnetic resonance elastography is able to detect cirrhosis with high specificity and an alkaline phosphatase <1.5 times the upper limit of normal makes the presence of advanced LS unlikely. Moreover, LS obtained by MRE is predictive of hepatic decompensation in PSC.
The only effective and approved therapy for chronic hepatitis C is interferon-alpha. Because sustained response rates with interferon alone are disappointingly low, multidrug treatment regimens are currently being investigated. Ursodeoxycholic acid has been used in other chronic liver diseases and can limit hepatocyte injury. To evaluate the potential benefit of ursodeoxycholic acid in combination with interferon-alpha for the treatment of chronic hepatitis C, we conducted a prospective, double-blinded, randomized, placebo-controlled trial comparing the combination therapy of interferon-alpha 2b and ursodeoxycholic acid with interferon alone. Thirty-one patients with chronic hepatitis C were randomized to receive 3 million units of interferon-alpha 2b subcutaneously three times per week and either 13 to 15 mg/kg/day ursodeoxycholic acid or placebo orally for 6 months. The 6-month treatment period was followed by 6 months of observation. Biochemical normalization at the end of treatment occurred in 5 of 14 (36%) patients receiving monotherapy versus 8 of 15 (53%) patients (p = 0.34) receiving combination therapy. No patient treated with interferon alone had a sustained biochemical response 6 months after therapy; however, 3 of 12 patients (25%) treated with combination interferon and ursodeoxycholic acid maintained biochemical normalization at 6 months after therapy (p = 0.08). No difference in liver histology or clearance of hepatitis C viral RNA was noted 6 months after treatment. We conclude that combination therapy with ursodeoxycholic acid and interferon-alpha 2b was no more effective than interferon monotherapy in inducing a biochemical response in previously untreated patients with chronic hepatitis C. Ursodeoxycholic acid, however, may be useful in prolonging the biochemical response to interferon therapy.
Autoimmune liver disease encompasses several disorders. Included in this category are primary sclerosing cholangitis (PSC), primary biliary cirrhosis (PBC), and autoimmune hepatitis (AIH). There are also several “overlap syndromes” which encompass two of these diseases in combination. In this chapter, we will describe these liver conditions, including the typical clinical presentation, the diagnostic criteria, management, and potential complications. We will also discuss briefly the role of orthotopic liver transplantation.
'/%3e%3c/g%3e%3cuse xlink:href='%23b' transform='matrix(1 0 0 -1 0 549)'/%3e%3c/svg%3e)