Introduction There are few data on presenting features of AIH outside large centres. We report data from an outcome audit of AIH in 28 UK centres of varying size.
Method We collected all prevalent cases since 2000 and all incident cases since 2007 by searching electronic patient letters, histology databases and hospital coding. Case validation was by 1999 International AIH Group diagnostic criteria. We used a web-based data collection system.
Results Of 1109 patients (227 prevalent 882 incident) with/treated as AIH, 80% were women. Age at diagnosis was (median (range)) 55 (8–86)years. Age distribution was similar in women and men. There were 91% Caucasian, 7% Asian, 1% Afro-Caribbean and 0.1% Chinese.39% had a personal/family history of autoimmune disease: 14% with thyroiditis/hypothyroidism, 10% PBC, 4% IBD and 3% coeliac disease. HBV and HCV serology was negative in 1061 (96%) patients and was undocumented in 42 (4%). Six patients (0.5%) had acute hepatitis IgM antibodies (4 CMV, 1 EBV and CMV, 1 HEV). ANA was present in 57%, ASMA in 47% and Anti-LKM1 in 2%; ≥1 one was found in 80%. AMA was found in 9%. Serum IgG/globulin was raised in 78%. Seventy four patients (7%) did not meet IAIHG diagnostic criteria but were treated as AIH.
Presenting symptoms were: none 22%, jaundice/itching 42%, fatigue 35%, nausea 14%, weight loss 13%, abdominal pain 16%, joint aches 12%, flu-symptoms 6%, rash 3%, amenorrhoea 0.4%, others 8%. Time from first abnormal liver tests to diagnosis was 3 (0–167), months and was shorter in patients presenting with jaundice/pruritus than in others: 1 vs 5 months; (p = < 0.0001). At presentation, 4.4% had ascites, 5.5% oedema, 1.9% encephalopathy, 0.7% variceal bleeding and 8.4% had clinical decompensation (≥1 of these). In another 13%, MELD score was >15. 96% of patients had a diagnostic liver biopsy of which 23% had cirrhosis (Ishak fibrosis stage 5/6).
Of 1030 treated patients (day one prescription) 1003 initially received a steroid: prednisolone (30 (2–60) mg/day) in 91%, budesonide (3–9 mg/day) in 5%, methylprednisolone in 1.6% and hydrocortisone in 0.1%. Of these, 11% received concurrent Azathioprine, 0.2% 6-Mercaptopurine (6-MP), 3% Mycophenolate, 2% Tacrolimus and 0.2% biological agents. Of those not receiving steroids initially (N = 27), 21 received AZA, 3 6 MP, 1 MMF, and 2 a biologic agent.
Conclusion In this large multicentre cohort of UK AIH patients, 23% had cirrhosis and 22% had liver decompensation at presentation. Delay in diagnosis was often many months, especially in those without jaundice. 4% did not have documented HBV/HCV serology and 7% did not meet IAIHG criteria. There is significant variation in initial treatment of AIH.
Disclosure of interest None Declared.