Population-based study of autoimmune hepatitis and primary biliary cholangitis in Germany: rising prevalences based on ICD codes, yet deficits in medical treatment

Abstract

Background Population-based data on the prevalence of and real-life treatment for the autoimmune liver diseases (AILD), primary biliary cholangitis (PBC) and autoimmune hepatitis (AIH), are scarce, and such knowledge may help to improve patient care. Methods Data of 8.1 million individuals having health insurance with the “Techniker Krankenkasse,” the largest German health insurer, were analyzed with regard to the prevalence of and prescribed medication for AILD in Germany from 2011 until 2014. Patients with viral hepatitis B infection (HBV) and alcoholic liver cirrhosis (ALC) served as control groups. Case definition was based on ICD codes. Results The prevalences of PBC and AIH were 36.9/100 000 inhabitants (95 % CI: 35.6–38.2) and 23.0/100 000 inhabitants (95 % CI: 22.0–24.0) in 2014, respectively. The prevalences of AILD increased from 2011 to 2014 (for PBC by 31 % and for AIH by 29 %), with the largest increase for male patients with PBC. In contrast, the prevalence of HBV declined while that of ALC remained stable. The analysis of prescribed real-life treatment revealed considerable deviations from standard treatment recommendations. Specifically, in older patients with PBC or AIH, undertreatment was common. Conclusion The prevalence of PBC and AIH based on ICD codes is increasing in Germany. The analysis of real-life treatment in this large and population-based cohort points to the unmet need to improve the implementation of treatment guidelines for autoimmune liver diseases in the broader medical community.

Global incidence and prevalence of autoimmune hepatitis, 1970–2022: a systematic review and meta-analysis

“…Table E6 shows the subgroup differences in incidence of AIH in this study. The pooled incidence of AIH was higher in countries with HDI >0.92 9 , 10 , 17 , 18 , 19 , 20 , 21 , 23 , 25 , 26 , 27 , 28 , 31 , 32 , 35 , 37 , 38 , 39 , 40 , 42 , 43 , 44 , 45 , 46 , 48 , 49 , 51 , 52 compared to countries with HDI <0.92, 24 , 34 , 36 , 41 with 1.63 (95% CI, 1.28–2.07) and 0.73 (95% CI, 0.49–1.08) cases per 100,000 inhabitant-years, respectively. The incidence of AIH in females 9 , 10 , 17 , 18 , 19 , 21 , 23 , 25 , 26 , 27 , 28 , 32 , 34 , 36 , 41 was higher than that in males, 9 , 10 , 17 , 18 , 19 , 21 , 23 , 25 , 26 , 27 , 28 , 32 , 34 , 36 , 41 ...…”

“…After evaluating 91 studies, 54 were excluded for reasons such as insufficient data on disease incidence, convenience sampling data, inappropriate study design, lack of population-based studies, and data duplication. Eventually, 37 studies (k = 37 9 , 10 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 ; researcher-validated studies, k = 21 9 , 21 , 26 , 27 , 28 , 30 , 31 , 34 , 35 , 36 , 37 , 38 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 48 , 49 ) were deemed eligible, involving 239,345,726 participants (approximately 2.9% of the global population) and 55,839 patients with AIH from 18 different countries (Argentina, Canada, Denmark, Finland, France, Iceland, Israel, Japan, New Zealand, Norway, Singapore, South Korea, Spain, Sweden, Taiwan, the Netherlands, the United Kingdom, and the United States [by country, 10.9% of the global population]) across five continents. Search strategy results are summarized in…”

“…All studies were conducted in a single country and included population-based studies on patients with AIH from 1970 to 2022. Twenty studies included both children and adults, 9 , 10 , 18 , 19 , 20 , 21 , 23 , 25 , 26 , 30 , 32 , 35 , 36 , 37 , 38 , 42 , 45 , 46 , 47 , 51 14 studies included only adults, 17 , 27 , 28 , 31 , 34 , 39 , 40 , 41 , 43 , 44 , 48 , 49 , 50 and 4 studies included only children. 24 , 29 , 33 , 52 Table 1 shows detailed characteristics of all the studies that met the inclusion criteria.…”

Characteristics and Long-term Outcome of 535 Patients with Histologically Confirmed Autoimmune Hepatitis (AIH) - A Single Center Experience of 18 Years

“…This is a long-term observational study over a time period of 18 years in a German cohort with a signi cant number of patients (n = 535) diagnosed with AIH [13][14][15]. Moreover, liver histology as a hallmark of the "International AIH-Score" was gained in each included patient.…”

Autoimmune Lebererkrankungen – ein Update (Teil 2)

“…Die Prävalenz der PBC wird mit 2 – 40/100 000 angegeben. Inzidenz und Prävalenz sind in den letzten Jahren deutlich zunehmend 2 .…”