Liver disease epidemiology and burden in patients with alterations in plasma protein metabolism: German retrospective insurance claims analysis

Abstract

BACKGROUND

Alpha-1 antitrypsin deficiency is a rare genetic disease and a leading cause of inherited alterations in plasma protein metabolism (APPM).

AIM

To understand the prevalence, burden and progression of liver disease in patients with APPM including alpha-1 antitrypsin deficiency.

METHODS

We conducted a retrospective analysis of anonymized patient-level claims data from a German health insurance provider (AOK PLUS). The APPM cohort comprised patients with APPM (identified using the German Modification of the International Classification of Diseases-10^th Revision [ICD-10-GM] code E88.0 between 01/01/2010-30/09/2020) and incident liver disease (ICD-10-GM codes K74, K70.2-3 and K71.7 between 01/01/2012-30/09/2020). The control cohort comprised patients without APPM but with incident liver disease. Outcomes were incidence/prevalence of liver disease in patients with APPM, demographics/baseline characteristics, diagnostic procedures, progression-free survival (PFS), disease progression and mortality.

RESULTS

Overall, 2680 and 26299 patients were included in the APPM (fibrosis, 96; cirrhosis, 2584) and control (fibrosis, 1444; cirrhosis, 24855) cohorts, respectively. Per 100000 individuals, annual incidence and prevalence of APPM and liver disease was 10-15 and 36-51, respectively. In the APPM cohort, median survival was 4.7 years [95% confidence interval (CI): 3.5-7.0] and 2.5 years (95%CI: 2.3-2.8) in patients with fibrosis and cirrhosis, respectively. A higher proportion of patients in the APPM cohort experienced disease progression (92.0%) compared with the control cohort (67.2%). Median PFS was shorter in the APPM cohort (0.9 years, 95%CI: 0.7-1.1) compared with the control cohort (3.7 years, 95%CI: 3.6-3.8; P < 0.001). Patients with cirrhosis in the control cohort had longer event-free survival for ascites, hepatic encephalopathy, hepatic failure and esophageal/gastric varices than patients with cirrhosis in the APPM cohort (P < 0.001). Patients with fibrosis in the control cohort had longer event-free survival for ascites, cirrhosis, hepatic failure and esophageal/gastric varices than patients with fibrosis in the APPM cohort (P < 0.001). In the APPM cohort, the most common diagnostic procedures within 12 mo after the first diagnosis of liver disease were imaging procedures (66.3%) and laboratory tests (51.0%).

CONCLUSION

Among patients with liver disease, those with APPM experience substantial burden and earlier liver disease progression than patients without APPM.

Keywords: Alpha-1 antitrypsin deficiency; Epidemiology; Germany; Liver diseases; Retrospective study

Core Tip: This was a retrospective analysis of anonymized, patient-level, insurance claims data from a German health insurance provider (AOK PLUS), which demonstrated that a diagnosis of alterations in plasma protein metabolism (APPM) (E88.0) in patients with liver disease was associated with a substantial burden and higher rate of liver disease progression compared with patients with liver disease but without APPM. To enable accurate diagnosis and inform disease management, it is important to have specific diagnostic codes that differentiate between genetic liver disease and liver manifestations from other causes.