The Deficiency of Serum Mannose Binding Lectin in Early Onset Idiopathic Pulmonary Fibrosis and Familial Cases

VA, Varney

doi:10.4172/2155-9899.1000127

Cited by 2 publications

(2 citation statements)

References 46 publications

(69 reference statements)

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“…The role of lectins in IPF is largely unknown. A previous study reported an association between MBL deficiency in serum and the development of IPF in young (<55 years) and familial cases of IPF, but not in older, typical IPF patients (12). The patients of our IPF cohort (mean age of 77) had rather elevated than deficient plasma MBL levels, thus confirming that MBL deficiency is probably not implicated in IPF.…”

Section: Discussionsupporting

confidence: 76%

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Local and Systemic Concentrations of Pattern Recognition Receptors of the Lectin Pathway of Complement in a Cohort of Patients With Interstitial Lung Diseases

et al. 2020

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Complement in Interstitial Lung Diseases p = 0.44; BALF, 37.5 vs. 24.6 ng/ml, p = 0.7) as well as lower ficolin-2 plasma levels (plasma 1111 vs. 1647 ng/ml, p = 0.11). Ficolin-2 plasma levels were inversely correlated with the forced vital capacity (r = 0.55, p = 0.1). Conclusion: This is the first study to simultaneously assess systemic and local lectin pathway protein levels in ILD patients. Our data suggest an involvement of PRR of the lectin pathway in the pathogenesis of sarcoidosis given the significantly higher BALF levels compared to a control group. Additional analyses in a larger patient cohort are required to confirm or refute a potential effect of local and/or systemic ficolin-2 levels in IPF patients.

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Section: Discussionsupporting

confidence: 76%

“…In the context of ILD, some evidence points to a relevant involvement of the lectin pathway. A case control study found a link between MBL deficiency and early onset IPF and familial cases (12). Similarly, MBL deficiency was associated with coal workers' pneumoconiosis, implying an important role of MBL in pulmonary host defense (13).…”

Section: Introductionmentioning

confidence: 99%

Local and Systemic Concentrations of Pattern Recognition Receptors of the Lectin Pathway of Complement in a Cohort of Patients With Interstitial Lung Diseases

et al. 2020

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Deficiency of Mannose Associated Serine Protease2 (MASP2) in Patients with Idiopathic Pulmonary Fibrosis

VA¹,

Parnell²

2015

J Clin Cell Immunol

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Background: Idiopathic pulmonary fibrosis (UIP/IPF) is increasing. The condition occurs both sporadically and in close blood relatives suggesting a genetic predisposition. Histology suggests that the fibrosis is linked to disordered apoptosis, but the causal agent is so far unrecognised. We previously published evidence of mannose binding lectin deficiency (MBL) in early onset disease and in those with a family history of UIP/IPF. MBL is part of the innate immune system with levels genetically determined. MBL activation is impaired if the mannose associated serine protease2 (MASP-2) is severely deficient in the serum (levels<100 ng/ml). The MBL/MASP-2 complex is involved in phagocytosis, complement activation and clearance of apoptotic cells. Levels of MASP-2 are genetically determined and not influenced by inflammation. Aims and Method: We examined plasma MASP-2 levels in healthy controls (HC) and in patients with frequently exacerbating COPD, pulmonary TB and sarcoid along with UIP/IPF including those with or without a family history. Results: Mean serum MASP-2 levels were lowest in those with UIP/IPF (304 ng/ml) compared with healthy controls (537 ng/ml), COPD (1090 ng/ml), TB (659 ng/ml) and sarcoid (385 ng/ml). Chi square analysis for the frequency of severe MASP-2 deficiency (<100 ng/ml versus >100 ng/ml) showed no differences between Healthy Controls, COPD, TB and sarcoid. UIP/IPF showed a significant increase in the frequency of severe deficiency. This was seen for early onset disease<55yrs (33% p=0.0001), late onset>55yrs (20% p=0.0001) and those with a family history (19% p=0.0143). The expected frequency for the Caucasian population is <1%. Conclusion: The data shows that severe MASP-2 deficiency is seen in our UIP/IPF patients and is highest in those with early onset disease. For TB and Sarcoid there is an increased frequency of deficiency (6-7%) compared with healthy controls (3%): and these diseases can also give fibrotic scarring to the lung. Since MASP-2 levels<100 ng/ml are considered to cause impaired function of the lectin pathway it could have relevance to complement activation, apoptosis and fibrosis in this disease.

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The Deficiency of Serum Mannose Binding Lectin in Early Onset Idiopathic Pulmonary Fibrosis and Familial Cases

Cited by 2 publications

References 46 publications

Local and Systemic Concentrations of Pattern Recognition Receptors of the Lectin Pathway of Complement in a Cohort of Patients With Interstitial Lung Diseases

Local and Systemic Concentrations of Pattern Recognition Receptors of the Lectin Pathway of Complement in a Cohort of Patients With Interstitial Lung Diseases

Deficiency of Mannose Associated Serine Protease2 (MASP2) in Patients with Idiopathic Pulmonary Fibrosis

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