Prevalence, Incidence, and Treatment Pattern of Wilson's Disease Using National Health Insurance Data From 2010-2020, Korea

Abstract

Background: Wilson's disease (WD) is an autosomal recessive disorder in which copper (Cu) accumulates in organs, particularly in the liver and central nervous system. This study aimed to investigate the prevalence, incidence, and treatment patterns of WD patients in Korea.

Methods: National Health Insurance System (NHIS) claims data from 2010 to 2020 were analyzed. patients with WD as a primary or additional diagnosis at least once were identified using the International Classification of Diseases (ICD)-10 disease code E83.0 and a record for a registration program for rare intractable diseases in Korea.

Results: The average age- and sex-adjusted prevalence and incidence of WD between 2010 and 2020 were 3.06/100,000 and 0.11/100,000, respectively. The mean age of the patients with newly diagnosed WD was 21.0 ± 15.9 years. Among the 622 WD incident cases during the study period, 19.3% of the patients had liver cirrhosis and 9.2% had received liver transplantation. Psychological and neurological diseases were present in 40.7% and 48.1% of the patients, respectively. Regarding the diagnosis of WD, liver biopsy was performed in only 51.6% of new cases. D-penicillamine, trientine, or zinc were prescribed in 81.5% of the incident cases, and the treatment uptake rates decreased with increasing age.

Conclusion: The prevalence of WD in Korea is 3.06/100,000 and approximately 1,800 patients use medical services annually. A significant proportion of patients are diagnosed at the cirrhotic stage and not treated with Cu-chelating therapeutics, suggesting the need for early diagnosis and adequate treatment to improve prognosis.

Keywords: Epidemiology; Penicillamine; Trientine; Wilson's Disease; Zinc.

Fig. 1. Prevalence (A) and incidence (B) of Wilson’s disease in South Korea during 2010–2020.

The bars indicate the number of patients with Wilson’s disease, and the line indicates the age- and sex-adjusted rates per 100,000 each year.

Fig. 2. Medical treatments for Wilson’s disease in South Korea during 2010–2020. (A) Drugs prescribed to patients with newly diagnosed Wilson’s disease in South Korea during 2010–2020. Numbers of patients treated with the drug (percentage of total incident cases). (B) Medical treatment uptake rates of patients with newly diagnosed Wilson’s disease according to the age group.