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Pulmonary alveolar proteinosis in Korea: analysis of prevalence and incidence via a nationwide population-based study.

ABSTRACT: BACKGROUND:Pulmonary alveolar proteinosis (PAP) is a very rare lung disease and its prevalence and incidence remain unclear. The prevalence and incidence of PAP were investigated by using nationwide claims data from the Korean Health Insurance Review and Assessment service. METHODS:Data were extracted for adults who visited any secondary or tertiary medical institute between 2010 and 2016 with the PAP-related Korean Classification of Disease, 7th edition code J84.0 and the Rare Intractable Disease exempted calculation code V222. To robust case definition, a narrow case definition was made when all following factors were met: 1) more than two PAP-coded visits within 1?year of the first claim, and 2) more than one claim for both chest computed tomography and diagnostic procedures (bronchoscopy or surgical lung biopsy) within 90?days before or after the first claim. RESULTS:A total of 182 patients (narrow, n?=?82) with PAP-related codes were identified from 2010 to 2016 and 89 new patients (narrow, n?=?66) visited medical institutes between 2012 and 2015. The prevalence of PAP was 4.44 (narrow: 2.27) per 106 population, with a peak age of 60-69?years. The incidence of PAP was 0.56 (narrow: 0.41) per 106 population at risk, with a peak age of 50-59?years. Among incident cases, the male-to-female ratio was 1.52 and about two-thirds had comorbidities, dyslipidaemia being the most common. CONCLUSIONS:The prevalence and incidence of PAP in Korea are low, similar to those in other countries; however, Korean patients with PAP are characterized by older diagnostic age and a lower male-to-female ratio.

SUBMITTER: Yoon HY

PROVIDER: S-EPMC7006195 | biostudies-literature | 2020 Feb

REPOSITORIES: biostudies-literature

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Pulmonary alveolar proteinosis in Korea: analysis of prevalence and incidence via a nationwide population-based study.

Yoon Hee-Young HY Kim Ji Hyeon JH Kim Ye-Jee YJ Song Jin Woo JW

BMC pulmonary medicine 20200206 1

<h4>Background</h4>Pulmonary alveolar proteinosis (PAP) is a very rare lung disease and its prevalence and incidence remain unclear. The prevalence and incidence of PAP were investigated by using nationwide claims data from the Korean Health Insurance Review and Assessment service.<h4>Methods</h4>Data were extracted for adults who visited any secondary or tertiary medical institute between 2010 and 2016 with the PAP-related Korean Classification of Disease, 7th edition code J84.0 and the Rare In ...[more]

PMID: 32028922

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Project description:AimsWe aim to determine the temporal trends of incidence, prevalence, and death of aortic stenosis (AS, I35.0) in an East Asian population.Methods and resultsData for 3773 patients who were newly diagnosed with AS from 2006 through 2017 were extracted from the National Health Insurance Service in Korea. The age-standardized incidence rate, prevalence rate, and death rate, survival rate (SR), and death risk of AS were calculated. Overall, the mean (standard deviation) age of AS patients was 69.9 (15.3) years [66.2 (15.7) years for men and 72.7 (14.4) years for women (P = 0.007)], and the proportion of men was 42.7%. The proportion of AS patients ≥60 years old was 80% (71.8% in men and 86% in women, P < 0.001). The proportion of patients who died of AS was 36.4% during the period from 2006 through 2017. The most common causes of death were disease of the circulatory system. The age-standardized incidence, prevalence, and death rates in 2017 were 0.85, 2.79, and 0.58 persons per 100 000 persons, respectively. The 10 year SR was 49.2%. The higher adjusted HRs [95% CI] for AS were observed in 70- to 79-year-old people (9.08 [1.27, 64.7], P = 0.027), in individuals 80 years of age or older (22.7 [3.18, 161.9], P = 0.001), in men (1.46 [1.31, 1.63], P < 0.001), among the middle socioeconomic group (1.19 [1.03, 1.37], P = 0.016), among the lower income levels (1.32 [1.17, 1.49], P < 0.001), in those with myocardial infarction (1.57 [1.16, 2.13], P = 0.003), with heart failure (1.63 [1.44, 1.85], P < 0.001), with ischaemic stroke (3.26 [1.20, 8.85], P = 0.015), with haemorrhagic stroke (2.51 [1.94, 3.25], P = 0.02), with chronic kidney disease (2.51 [1.94, 3.25], P < 0.001), and with malignant neoplasm (2.33 [1.64, 3.31], P < 0.001).ConclusionsThe proportion of AS at age ≥60 years was 80%. For AS, the age-standardized incidence rates were steady, prevalence rates increased, and death rates decreased by year over a decade. The 10 year SR of AS was about 50%. The most common cause of death in AS was disease of the circulatory system. Given the progressively higher incidence of AS with age continued efforts are required to increase awareness regarding AS-related symptoms and potential complications in aged people.

Project description:BackgroundAtherosclerotic cardiovascular disease (ASCVD) is the leading cause of death in Korea. According to a report of published by Statistics Korea in 2014, cerebrovascular disease and cardiovascular disease were the major/leading causes of mortality. However, it is more difficult to identify prevalence and incidence of a disease than the mortality owing to the lack of national-level statistics. Few studies have examined the prevalence and incidence of ASCVD and its risk factors since 2012. This study aimed to estimate the prevalence and incidence of ASCVD and its risk factors in Korea using national claims data.MethodsWe conducted a retrospective analysis using the national claims data of the Health Insurance Review and Assessment Service. Patients aged ≥18 years with ASCVD (defined as myocardial infarction, angina, coronary revascularization, peripheral artery disease, ischemic stroke, and transient ischemic attack) were identified between January 1, 2014 and December 31, 2015. Patients at high risk for ASCVD (defined as hypertension, diabetes mellitus, and dyslipidemia without ASCVD during the baseline period) were identified between January 1, 2015 and December 31, 2015. We estimated the prevalence, cumulative incidence, and incidence density. These were further stratified by age and sex. The respective denominators for prevalence and incidence were the census population and the at-risk population (defined as the population without respective disease 1 year prior to the respective disease identification).ResultsAmong the included Korean adult patients, the overall prevalence of clinical ASCVD per 1000 individuals was 98.25 in 2014 and 101.11 in 2015. The respective cumulative incidence and incidence density rates of ASCVD per 1000 individuals were 65.30 and 68.03 in 2014, and 67.05 and 69.94 in 2015, respectively. Peripheral artery disease seemed to drive the increase in the total prevalence and incidence of ASCVD. The prevalence and incidence of ASCVD continued to increase with age until 79 years.ConclusionsThis national population-based study confirmed the high prevalence and incidence of ASCVD and its risk factors in the adult population of South Korea. We suggest that more intensive treatment and prevention are needed to prevent ASCVD.

Project description:BackgroundPulmonary alveolar proteinosis (PAP) is a rare clinical syndrome involving the accumulation of lipid-rich proteinaceous material in the alveoli. There is a paucity of published studies on this condition. To better characterize the demographics, complication rates, mortality, and healthcare costs of patients hospitalized for PAP in the United States, a secondary analysis on the Hospital Cost and Utilization Project's Nationwide Inpatient Sample (NIS) was performed on patients admitted from 2012 to 2014 with a diagnosis of pulmonary alveolar proteinosis.MethodsUsing the NIS database, a secondary analysis was performed on 500 admissions with the diagnosis "pulmonary alveolar proteinosis." The clinical variables and outcome measures extracted were: patient demographics, hospital costs, length of stay, frequency of admissions, and inpatient mortality rate.ResultsAmong a weighted estimate of 500 hospital admissions from 2012 to 2014, the number of PAP admissions averaged 4.7 per million. The population was predominantly male (55%) with a mean age of 41.45 (CI 38.3-44.5) from all socioeconomic levels. Inpatient mortality was calculated to be 5%, which may result from the fact that the majority of admitted patients had few or no comorbid conditions (CCI 0.72). The most common procedure performed during admission was a bronchoalveolar lavage. Mean length of stay was 6.2 days (CI 3.9-8.5) and average cost of admission was $29,932.20 (CI 13,739-46,124). Of note, 50% of these admissions were considered "elective."ConclusionsDemographics of patients with PAP who have been hospitalized in the United States are similar to previously reported demographics from prior patient cohorts, specifically a male predominance and a mean age in the 40 s. The inpatient mortality rate of 5% we found is consistent with prior studies demonstrating good disease-specific survival rates. Notably, the cost per admission and overall annual cost associated with PAP hospitalization was calculated to be $29932.20 and $5 million respectively. This reflects the high economic cost associated with hospitalization of PAP patients, and provokes thought about ways to make treatment more cost-effective.

Project description:Pulmonary Alveolar Proteinosis (PAP) patients exhibit an acquired deficiency of biologically active granulocyte-macrophage colony stimulating factor (GM-CSF) attributable to GM-CSF specific autoantibodies. PAP alveolar macrophages are foamy, lipid-filled cells with impaired surfactant clearance and markedly reduced expression of the transcription factor peroxisome proliferator-activated receptor gamma (PPAR?) and the PPAR?-regulated ATP binding cassette (ABC) lipid transporter, ABCG1. An open label proof of concept Phase II clinical trial was conducted in PAP patients using rituximab, a chimeric murine-human monoclonal antibody directed against B lymphocyte specific antigen CD20. Rituximab treatment decreased anti-GM-CSF antibody levels in bronchoalveolar lavage (BAL) fluid, and 7/9 patients completing the trial demonstrated clinical improvement as measured by arterial blood oxygenation.This study sought to determine whether rituximab therapy would restore lipid metabolism in PAP alveolar macrophages.BAL samples were collected from patients pre- and 6-months post-rituximab infusion for evaluation of mRNA and lipid changes.Mean PPAR? and ABCG1 mRNA expression increased 2.8 and 5.3-fold respectively (p???0.05) after treatment. Lysosomal phospholipase A2 (LPLA2) (a key enzyme in surfactant degradation) mRNA expression was severely deficient in PAP patients pre-treatment but increased 2.8-fold post-treatment. In supplemental animal studies, LPLA2 deficiency was verified in GM-CSF KO mice but was not present in macrophage-specific PPAR? KO mice compared to wild-type controls. Oil Red O intensity of PAP alveolar macrophages decreased after treatment, indicating reduced intracellular lipid while extracellular free cholesterol increased in BAL fluid. Furthermore, total protein and Surfactant protein A were significantly decreased in the BAL fluid post therapy.Reduction in GM-CSF autoantibodies by rituximab therapy improves alveolar macrophage lipid metabolism by increasing lipid transport and surfactant catabolism. Mechanisms may involve GM-CSF stimulation of alveolar macrophage ABCG1 and LPLA2 activities by distinct pathways.

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Pulmonary alveolar proteinosis in Korea: analysis of prevalence and incidence via a nationwide population-based study.

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Pulmonary alveolar proteinosis in Korea: analysis of prevalence and incidence via a nationwide population-based study.

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