A Multidimensional Index and Staging System for Idiopathic Pulmonary Fibrosis

Ley, Brett; Ryerson, Christopher J.; Vittinghoff, Eric; Ryu, Jay H.; Tomassetti, Sara; Lee, Joyce; Poletti, Venerino; Buccioli, Matteo; Elicker, Brett M.; Jones, Kirk D.; King, Talmadge E.; Collard, Harold R.

doi:10.7326/0003-4819-156-10-201205150-00004

Cited by 1,051 publications

(1,026 citation statements)

References 25 publications

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“…Future studies should also consider whether sLOXL2, along with other promising prognostic IPF biomarkers (e.g. matrix metalloproteinase (MMP)1, MMP7, KL-6, periostin, surfactant protein-A and D, CC chemokine ligand 18, vascular endothelial growth factor and YKL-40) [19][20][21][22][23][24][25][26][27][28][29], as well as prognostic scores [30,31] and radiological modalities [32], might have prognostic value for helping physicians and patients anticipate the patient's IPF disease progression. This might include evaluation of serially collected sLOXL2 levels and their relationship to IPF acute exacerbations, which represent a terminal event for many IPF patients [33].…”

Section: Discussionmentioning

confidence: 99%

Serum lysyl oxidase-like 2 levels and idiopathic pulmonary fibrosis disease progression

Chien¹,

et al. 2013

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We evaluated whether lysyl oxidase-like 2 (LOXL2), which promotes cross-linking of collagen in pathological stroma, was detectable in serum from idiopathic pulmonary fibrosis (IPF) patients, and assessed its relationship with IPF disease progression.Patients from the ARTEMIS-IPF (n569) and the Genomic and Proteomic Analysis of Disease Progression in IPF (GAP) (n5104) studies were analysed. Baseline serum LOXL2 (sLOXL2) levels were compared with baseline clinical and physiological surrogates of disease severity, and the association with IPF disease progression was assessed using a classification and regression tree (CART) method. sLOXL2 correlated weakly with forced vital capacity and carbon monoxide diffusion capacity (r -0.24-0.05) in both cohorts. CART-determined thresholds were similar: ARTEMIS-IPF 800 pg?mL -1 and GAP 700 pg?mL -1. In ARTEMIS-IPF, higher sLOXL2 (.800 pg?mL -1 ) was associated with increased risk for disease progression (hazard ratio (HR) 5.41, 95% CI 1.65-17.73). Among GAP subjects with baseline spirometric data (n570), higher sLOXL2 levels (.700 pg?mL -1 ) were associated with more disease progression events (HR 1.78, 95% CI 1.01-3.11). Among all GAP subjects, higher sLOXL2 levels were associated with increased risk for mortality (HR 2.28, 95% CI 1.18-4.38).These results suggest that higher sLOXL2 levels are associated with increased risk for IPF disease progression. However, due to multiple limitations, these results require validation. Clinical trial: This study is registered at www.ClinicalTrials.gov with identifier number NCT00768300 and NCT 00373841.

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Section: Discussionmentioning

confidence: 99%

Serum lysyl oxidase-like 2 levels and idiopathic pulmonary fibrosis disease progression

Chien¹,

et al. 2013

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“…Ley and colleagues found that GAP index correlated well with 1-year mortality among patients with IPF [29,30]. …”

Section: Methodsmentioning

confidence: 99%

Validation of the COPD Assessment Test (CAT) in patients with idiopathic pulmonary fibrosis

Grufstedt

Shaker

Konradsen

2018

European Clinical Respiratory Journal

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Introduction: Idiopathic pulmonary fibrosis (IPF) is a chronic disease with high morbidity and mortality. A reliable and manageable questionnaire that assesses patients’ quality of life is needed to help to improve the content of clinical consultations. The chronic obstructive pulmonary disease (COPD) Assessment Test (CAT) is used in clinical practice; therefore, the aim of this study was to investigate correlations among the CAT, St. George’s Respiratory Questionnaire (SGRQ) and clinical parameters among patients diagnosed with IPF.Methods: A retrospective cohort design was employed in which 87 patients diagnosed with IPF who were receiving treatment at an outpatient clinic were included.Results: The CAT was found to be significantly correlated with SGRQ (p < 0.001). Furthermore, the CAT was significantly correlated with all of the included physiological variables.Conclusions: This add to support the validity of the COPD Assessment test (CAT) as a measure of symptoms in patients with IPF which in clinical practice in combination with dialogue between healthcare professionals and the patient, can help to improve the content of a clinical consultation.

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“…More recently, LEY et al [22] developed a multidimensional risk prediction model and staging system using data from three large, geographically distinct cohorts of IPF patients in California (USA), Minnesota (USA) and Northern Italy. This GAP model consists of four baseline variables: gender (G), age (A) and two lung physiology variables (P) (FVC and DLCO).…”

Section: Novel Ipf Staging Systemsmentioning

confidence: 99%

Staging of idiopathic pulmonary fibrosis: past, present and future

Kolb

Collard

2014

Eur Respir Rev

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Idiopathic pulmonary fibrosis (IPF) is traditionally staged with terms such as ''mild'', ''severe'', ''early'' and ''advanced'' based on pulmonary function tests. This approach allows physicians to monitor disease progression and advise patients and their families. However, it is not known if the stages of this model reflect distinct biological or clinical phenotypes and the therapeutic and prognostic value of this system is limited.Novel methods of IPF staging have recently been developed. The GAP model includes four baseline variables that were found to be predictive of outcome, as identified by logistic regression. These factors are: gender (G), age (A) and two lung physiology variables (P) (forced vital capacity and diffusing capacity of the lung for carbon monoxide). The clinical utility and accuracy of staging models may be further improved in the future by the integration of dynamic parameters that can be measured over time, as well as biological data from biomarkers which may be able to directly measure disease activity. The development of an evidence-based, multidimensional IPF staging model that builds on the current staging approaches to IPF is an important objective for improving the management of IPF. @ERSpublications Development of an evidence-based, multidimensional IPF staging model is important for improving IPF management

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A Multidimensional Index and Staging System for Idiopathic Pulmonary Fibrosis

Abstract: The GAP models use commonly measured clinical and physiologic variables to predict mortality in patients with IPF.

Cited by 1,051 publications

References 25 publications

Serum lysyl oxidase-like 2 levels and idiopathic pulmonary fibrosis disease progression

Serum lysyl oxidase-like 2 levels and idiopathic pulmonary fibrosis disease progression

Validation of the COPD Assessment Test (CAT) in patients with idiopathic pulmonary fibrosis

Staging of idiopathic pulmonary fibrosis: past, present and future

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