Impaired lipid metabolism in idiopathic pulmonary alveolar proteinosis

Tian, Xinlun; Luo, Jinmei; Xu, Kai‐Feng; Wang, Lan; Zhou, Jiong; Feng, Ru; Gui, Yao-Song; Wang, Juan; Xu, Weifeng; Xiao, Yi; Zhu, Ying

doi:10.1186/1476-511x-10-54

Cited by 8 publications

(11 citation statements)

References 28 publications

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“…Patients with iPAP exhibited elevated lipid levels that were reduced significantly after whole lung lavage. In addition, the present study supports the view that impaired lipid metabolism could be present in iPAP[1,6]. These findings may expand the role of serum lipid levels in iPAP.…”

Section: Discussionsupporting

confidence: 90%

“…Several studies have indicated that circulating lipoprotein levels would be changed in iPAP[ 1 , 5 , 6 ]. Elevated cholesterol levels have been described in 19% of the PAP patients, however, sample size and characteristics were not available[ 1 ].…”

Section: Discussionmentioning

confidence: 99%

“…It has been reported[ 6 ] that serum levels of triglyceride were higher in patients with idiopathic PAP, while HDL-C levels were lower in patients. Similarly, serum levels of cholestenoic acid were also significantly increased in the PAP patients[ 7 ].…”

Section: Introductionmentioning

confidence: 99%

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Clinical significance of serum lipids in idiopathic pulmonary alveolar proteinosis

et al. 2012

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BackgroundIt is well known that pulmonary alveolar proteinosis(PAP) is characterised by accumulation of surfactant lipids and proteins within airspaces. However, few previous data describe the serum lipid levels associated with PAP.Materials and methodsWe retrospectively reviewed 25 patients with idiopathic PAP(iPAP). The serum lipid levels of patients with idiopathic PAP were compared with those of the healthy volunteers. In patients and healthy subjects, the LDL-C/HDL-C ratios were 2.94 ± 1.21 and 1.60 ± 0.70, respectively (p < 0.001), HDL-C were 1.11 ± 0.27 and 1.71 ± 0.71 respectively (p < 0.001). The values of LDL-C correlated significantly with those of PaO2 and PA-aO2 (r = -0.685, p = 0.003, and r = 0.688, p = 0.003, respectively). The values of LDL-C/HDL-C ratios also correlated with PaO2 levels and PA-aO2 levels (r = -0.698, p = 0.003, and r = 0.653, p = 0.006, respectively). 11 and 13 patients experienced respectively a decline in TC and LDL-C levels following whole lung lavage(WLL), the median decline was 0.71 mmol/L(p < 0.009) and 0.47 mmol/L(p < 0.003), respectively.Conclusionsthe serum lipid levels, especially the levels of LDL-C and LDL-C/HDL-C, may reflect the severity of the disease in PAP patients, and predict the therapeutic effect of WLL.

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Section: Discussionsupporting

confidence: 90%

Section: Discussionmentioning

confidence: 99%

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Clinical significance of serum lipids in idiopathic pulmonary alveolar proteinosis

et al. 2012

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“…In the Korean population, the prevalence of hypercholesterinaemia, hypertriglyceridemia, and diabetes were reported to be 19.9, 17.2, and 11.3%, respectively in 2016, suggesting an increased prevalence of metabolic disorders in Korean patients with PAP when compared with the general population. Previous studies support our findings; in a study involving non-obese and non-diabetic PAP patients (n = 33), Tian et al, [21]. This finding might be linked to the cholesterol-lowering effect of GM-CSF by promoting macrophage functions in lipid metabolism [22].…”

Section: Discussionsupporting

confidence: 89%

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

Yoon

Kim

et al. 2020

BMC Pulm Med

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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 10 6 population, with a peak age of 60-69 years. The incidence of PAP was 0.56 (narrow: 0.41) per 10 6 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.

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“…Our results do not exclude the possibility that effects of statin therapy on serum cholesterol and HDL may contribute to therapeutic efficacy in PAP. Indeed, autoimmune PAP patients have an increased serum LDL:HDL ratio and reduced HDL levels 39 , 40 and the LDL to HDL ratio is inversely correlated with disease severity 39 . This study did not define the optimal dose of statin therapy or the time required to reach maximal treatment effect and additional studies are needed to further determine the specific mechanism of action, pharmacokinetics, and the potential role of statin therapy in autoimmune PAP.…”

Section: Discussionmentioning

confidence: 99%

Statin as a novel pharmacotherapy of pulmonary alveolar proteinosis

et al. 2018

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Pulmonary alveolar proteinosis (PAP) is a syndrome of reduced GM-CSF-dependent, macrophage-mediated surfactant clearance, dysfunctional foamy alveolar macrophages, alveolar surfactant accumulation, and hypoxemic respiratory failure for which the pathogenetic mechanism is unknown. Here, we examine the lipids accumulating in alveolar macrophages and surfactant to define the pathogenesis of PAP and evaluate a novel pharmacotherapeutic approach. In PAP patients, alveolar macrophages have a marked increase in cholesterol but only a minor increase in phospholipids, and pulmonary surfactant has an increase in the ratio of cholesterol to phospholipids. Oral statin therapy is associated with clinical, physiological, and radiological improvement in autoimmune PAP patients, and ex vivo statin treatment reduces cholesterol levels in explanted alveolar macrophages. In Csf2rb−/− mice, statin therapy reduces cholesterol accumulation in alveolar macrophages and ameliorates PAP, and ex vivo statin treatment increases cholesterol efflux from macrophages. These results support the feasibility of statin as a novel pathogenesis-based pharmacotherapy of PAP.

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Impaired lipid metabolism in idiopathic pulmonary alveolar proteinosis

Cited by 8 publications

References 28 publications

Clinical significance of serum lipids in idiopathic pulmonary alveolar proteinosis

Clinical significance of serum lipids in idiopathic pulmonary alveolar proteinosis

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

Statin as a novel pharmacotherapy of pulmonary alveolar proteinosis

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