A novel serum metabolomic panel distinguishes IgG4-related sclerosing cholangitis from primary sclerosing cholangitis.

BACKGROUND & AIMS: Primary sclerosing cholangitis (PSC) and IgG4-related sclerosing cholangitis (IgG4-SC) are chronic fibro-inflammatory immune-mediated hepatobiliary conditions that are challenging to distinguish in a clinical setting. Accurate non-invasive biomarkers for discriminating PSC and IgG4-SC are important to ensure a correct diagnosis, prompt therapy and adequate cancer surveillance. METHODS: We performed nuclear magnetic resonance (NMR)-based metabolomic profiling using serum samples collected prospectively from patients with PSC (n = 100), IgG4-SC (n = 23) and healthy controls (HC; n = 16). RESULTS: Multivariate analysis of the serum metabolome discriminated PSC from IgG4-SC with greater accuracy (AUC 0.95 [95%CI 0.90-0.98]) than IgG4 titre (AUC 0.87 [95%CI 0.79-0.94]). When inflammatory bowel disease (IBD) was excluded as a comorbid condition (IgG4-SC n = 20, PSC n = 22), the diagnostic AUC increased to 1.0, suggesting that the metabolome differences identified are not a result of the increased prevalence of IBD in PSC relative to IgG4-SC patients. Serum lactate (p < .0001), glucose (p < .01) and glutamine (p < .01) metabolites were increased in IgG4-related disease (IgG4-RD) and IgG4-SC individuals compared to PSC, whereas mobile choline (p < .05), 3-hydroxybutyric acid (p < .01) and -CH3 lipoprotein resonances (p < .01) were decreased. CONCLUSIONS: Taken together, serum metabolomic profiling has the potential to be incorporated as a diagnostic criterion, independent of IgG4 titre, to improve the diagnosis of IgG4-RD and help distinguish IgG4-SC from PSC.

Upper gastrointestinal tract involvement in Crohn's disease: A case report.

BACKGROUND: Crohn's disease (CD) is an inflammatory bowel disease that typically affects the distal part of the gastrointestinal tract (GI) such as the terminal ileum and colon. However, it can affect the upper GI tract, potentially resulting in complications such as strictures, but discussion of the management of such effects is limited in the surgical literature. CASE PRESENTATION: A 39 year old male was referred to our department with stricturing upper GI disease 20 years after CD diagnosis. He had a history of intermittent abdominal pain, nausea, frequent vomiting and weight loss. Imaging demonstrated a long stricture in the duodenum with proximal dilatation. There was no evidence of acute inflammatory Crohn's disease. A Roux-en-Y bypass was performed to successfully relieve the obstructive symptoms. DISCUSSION: Proximal obstructive gastrointestinal manifestations of CD are a rare entity and require a full diagnostic workup and treatment in a specialist centre. A variety of systemic treatments, endoscopic procedures and surgical techniques are addressed in this paper. CONCLUSION: Evidence for the optimal treatment of obstructive upper gastrointestinal CD is limited, but careful consideration of the extent of the disease, thorough preoperative planning and weighing up the benefits and risks can lead to a positive outcome for these patients.