The communication of a secondary care diagnosis of autoimmune hepatitis to primary care practitioners: a population-based study

Background Autoimmune Hepatitis is a chronic liver disease which affects young people and can result in liver failure leading to death or transplantation yet there is a lack of information on the incidence and prevalence of this disease and its natural history in the UK. A means of obtaining this information is via the use of clinical databases formed of electronic primary care records. How reliably the diagnosis is coded in such records is however unknown. The aim of this study therefore was to assess the proportion of consultant hepatologist diagnoses of Autoimmune Hepatitis which were accurately recorded in General Practice computerised records. Methods Our study population were patients with Autoimmune Hepatitis diagnosed by consultant hepatologists in the Queens Medical Centre, Nottingham University Hospitals (UK) between 2004 and 2009. We wrote to the general practitioners of these patients to obtain the percentage of patients who had a valid READ code specific for Autoimmune Hepatitis. Results We examined the electronic records of 51 patients who had biopsy evidence and a possible diagnosis of Autoimmune Hepatitis. Forty two of these patients had a confirmed clinical diagnosis of Autoimmune Hepatitis by a consultant hepatologist: we contacted the General Practitioners of these patients obtaining a response rate of 90.5% (39/42 GPs). 37/39 of these GPs responded with coding information and 89% of these patients (33/37) used Read code J638.00 (Autoimmune Hepatitis) to record a diagnosis. Conclusions The diagnosis of Autoimmune Hepatitis made by a Consultant Hepatologist is accurately communicated to and electronically recorded by primary care in the UK. As a large proportion of cases of Autoimmune Hepatitis are recorded in primary care, this minimises the risk of introducing selection bias and therefore selecting cases using these data will be a valid method of conducting population based studies on Autoimmune Hepatitis

Diabetes mellitus in patients with autoimmune hepatitis: frequency, risk factors and effect on outcome

Background Treatment for autoimmune hepatitis (AIH) includes corticosteroids, which are associated with the development of diabetes mellitus (DM). Reported new-onset DM rates in patients with AIH have varied, and predisposing factors and prognostic implications are inadequately characterised. Aim To identify the frequency and predisposing factors for DM in AIH and its association with disease progression and mortality. Methods Retrospective/prospective single-centre study of 494 patients with AIH presenting 1987–2023, 466 receiving corticosteroids (454 prednisolone, 12 budesonide) and followed for (median (range) 9 (0–36) years). Results Forty-seven patients (10%) already had DM at AIH diagnosis. New-onset DM subsequently developed in another 59 (13%). In those receiving prednisolone, new-onset DM incidence was 8% ± 1% after 1 year and 14% ± 2% after 10 years (14- and 3-fold higher than expected population rate), and was independently associated with older age, non-Caucasian ethnicity, higher initial prednisolone dose, higher BMI at diagnosis and more weight gain after 2 years of follow-up. New-onset DM usually persisted despite stopping prednisolone. New-onset DM and DM at any time were independently associated with all-cause death/transplantation rate, along with previously established risk factors (older age, cirrhosis, lower ALT at diagnosis and failure of early ALT normalisation). New-onset DM and DM at any time were also independently associated with cirrhosis development. Similar associations of new-onset DM and DM at any time with liver-related death/transplantation were significant on univariate but not multivariate analysis. Conclusion New-onset DM occurred in 13% of patients with AIH, was related to older age, non-Caucasian ethnicity, higher prednisolone dose, higher BMI at diagnosis and weight gain; and was an independent predictor of all-cause death/transplantation and of cirrhosis development, underlining the need to minimise steroid burden in AIH

International genome-wide meta-analysis identifies new primary biliary cirrhosis risk loci and targetable pathogenic pathways

Primary biliary cirrhosis (PBC) is a classical autoimmune liver disease for which effective immunomodulatory therapy is lacking. Here we perform meta-analyses of discovery data sets from genome-wide association studies of European subjects (n1⁄42,764 cases and 10,475 controls) followed by validation genotyping in an independent cohort (n1⁄43,716 cases and 4,261 controls). We discover and validate six previously unknown risk loci for PBC (Pcombinedo5108) and used pathway analysis to identify JAK-STAT/IL12/IL27 signalling and cytokine–cytokine pathways, for which relevant therapies exist