C Sikavi et al. Hepatology 2018; 67: 847-57. This systematic review highlights that the combination of hepatitis C virus (HCV) infection and HIV infection is no longer a difficult-to-treat population with the implementation of direct-acting antivirals (DAAs). There are similar sustained virologic responses (SVRs) among those with and those without HIV. In clinical trials, patients with combined HCV-HIV had SVRs of 93.5-98% with DAA treatment; “real-world cohorts” had SVRs of 90.9%-98%.
MS Middleton et al. Hepatology 2018; 67: 858-72. Using data from the prospective CyNCh trial (cysteamine for NAFLD), the authors examined MRIs for diagnostic accuracy among 169 enrolled children. In this group, 110 (65%) and 83 (49%) had MRI and liver biopsy at baseline. MRI-PDFF (proton density fat fraction) was able to classify grade 1 steatosis from grade 2-3 steatosis with area under receiving operator characteristic curve of 0.87. Thus, this study shows MRI-estimated PDFF has high diagnostic accuracy.
G Mieli-Vergani et al. JPGN 2018; 66: 345-60. Position paper for Pediatric Autoimmune Liver Disease (AIH, ASC, de novo AIH after liver transplantation). This is a very useful review. A couple of pointers from the authors:
- “Present experience with budesonide as the first-line treatment is limited and does not appear to offer clear clinical advantage over the standard treatment”[prednisone]
- Fecal calprotectin should be obtained to evaluate for IBD in patients with autoimmune liver disease, “even in asymptomatic children.”
JM Cotter et al. JPGN 2018; 66: 227-33. This retrospective study with 39 patients with primary sclerosing cholangitis (PSC) showed a lack of correlation between liver tests and fibrosis at presentation. Average age of PSC diagnosis was 11.2 years, 74% had inflammatory bowel disease and 51% had autoimmune hepatitis. Related blog post: Big Pediatric PSC Study (with 781 children)
A recent retrospective study (S Jitaruch et al. J Pediatr 2017; 189: 79-85) documents the important association of autoimmune liver disease in children with sickle cell disease (SCD). I have seen children with hemoglobinopathy and autoimmune hepatitis.
- 13 of 77 patients with SCD were diagnosed with autoimmune liver disease
- 2 patients presented with acute liver failure
- 6 patients had cholangiopathy on cholangiogram “suggesting autoimmune sclerosing cholangitis”
- At median follow-up of 3.8 years, 5 achieved full remission, 4 partial remission, 1 had liver transplant, 1 died of subdural hemorrhage (prior to liver disease treatment), and 2 were lost to followup
My take: this report is a good reminder that though there are a good number of reasons for abnormal liver blood tests in children with SCD, it is important to follow these children closely and to obtain serology (including ANCA) in those with persistent elevations.
Related blog post: Blood is not enough
South Kaibab Trail at the basin of the Grand Canyon (Colorado River)
A recent review (Full text: LJ Saubermann et al. JPGN 2017; 64: 639-52) discusses the hepatic issues and complications associated with inflammatory bowel disease.
- Primary Sclerosing Cholangitis (PSC)
- Autoimmune Hepatitis (AIH)
- Autoimmune Sclerosing Cholangitis (ASC)
- Portal Venous Thrombosis/hypercoagulability
- Cholelithiasis (more common in Crohn’s disease if diseased terminal ileum)
- Viral hepatitis
- Drug-Induced Liver Disease
- Fatty Liver disease
Many of these topics have been discussed previously on this blog. A couple of pointers in this review:
- Greater risk of colorectal carcinoma
- IBD-PSC patients are at higher risk for pouchitis
- GGT of >252 U/L “was highly sensitive (99%) and had good specificity (71%) for PSC” [or ASC]
- The authors recommend “screening all newly diagnosed patients with IBD with ALT and GGT
- Immunosuppressive therapy is NOT effective
- Vancomycin therapy is currently being tested (clinical trials: NCT02137668 & NCT01802073)
- Less frequent in IBD patients than PSC
- Most common treatment is prednisone/azathioprine
- 40-80% of children have cirrhosis at AIH diagnosis, but “progression to end-stage liver disease is rare and …with appropriate treatment, 80% of patients achieve remission.”
- ASC is an overlap syndrome between AIH and PSC
- “It is important that children with IBD and apparent AIH are routinely investigated for evidence of biliary disease with MRCP”
- “ASC responds to the same immunosuppressive combination therapy used for AIH”
- HAV vaccination is effective in patients with IBD…although the rate [seroconversion] was significantly lower” in patients receiving anti-TNF therapy (92.4% vs 99.1% in one study).
- In those needing HBV immunization: “One strategy evaluated to improve HBV immunity in adults with IBD is an accelerated course with double vaccine doses at 0, 1, and 2 months.”
- “The extent of histological features of hepatotoxicity secondary to long-term MTX use in IBD has been infrequently described; however, the inicdence of significant abnormal histological findings appears to be rather low.”
My take: This article is a good starting point for liver-related issues in IBD. For concerns regarding medications, the NIH livertox website is more useful and much more comprehensive.
Related blog entries:
RJ Fontana et al. Hepatology 2016; 64: 1870-80. In this study of 681 adults with acute liver failure in U.S., only 3 had detectable anti-HEV IgM and all three were negative for HEV-RNA. In addition, other putative causes of acute liver failure were present in all three. My take: Hepatitis E is very rare explanation for acute liver failure in the U.S.
RA Rosencrantz et al. Hepatology 2016; 64: 2253-6. Case report of 2.5 yr old with autoimmune sclerosing cholangitis with Kawasaki disease. This was a well-described case with MRCP and liver histology. My take: In patients with Kawasaki with protracted liver disease, another etiology to consider.
Related blog posts: