Mediastinal Lymph Node Enlargement Predicts Significant Functional Decline in Idiopathic Pulmonary Fibrosis

Abstract Background and objectives Evidence of mediastinal Lymph Node Enlargement (LNE) on CT scan is a common finding in idiopathic pulmonary fibrosis (IPF). We sought to investigate whether the involvement of mediastinal lymph nodes is associated with accelerated disease progression, and explored the changes occurring in mediastinal lymph nodes during the radiological follow up of these patients. Methods This retrospective study included IPF patients referred to a single ILD centre in Italy. A consensus-based assessment of mediastinal LNE on chest CT scan was performed by two thoracic radiologists. Kaplan-Meier curves and multivariate Cox proportional hazards regression were used to assess hazard ratios for mortality and disease progression (defined as categorical FVC decline ≥10%). The annualized rates of change in functional parameters for each patient were calculated using mixed linear models. Results The study population consisted of 152 IPF patients, of whom 135 (89%) received antifibrotic treatment for IPF during the study follow up. Patients having evidence of 3 or more enlarged mediastinal lymph nodes on baseline CT scan showed increased rates of mortality (HR 5.03, 95% CI 1.86–13.62, p ≤ 0.001) and significant disease progression (HR 2.99, 95% CI 1.22–7.33, p = 0.17) as compared to patients without LNE, after adjusting for GAP stage. Among 62 patients with LNE who underwent a follow up CT scan of the chest and received antifibrotic treatment, 57 (92%) maintained evidence mediastinal LNE over time. Conclusions Diffuse mediastinal lymph node involvement predicts clinically meaningful functional deterioration in patients with IPF.

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Mediastinal lymph node enlargement in idiopathic pulmonary fibrosis: relationships with disease progression and pulmonary function trends.

10.21203/rs.3.rs-46826/v2 ◽

2020 ◽

Author(s):

Giacomo Sgalla ◽

Anna Rita Larici ◽

Nicoletta Golfi ◽

Mariarosaria Calvello ◽

Alessandra Farchione ◽

...

Keyword(s):

Lymph Node ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Lymph Nodes ◽

Ct Scan ◽

Disease Progression ◽

Mediastinal Lymph Node ◽

Mediastinal Lymph Nodes ◽

Lymph Node Enlargement

Abstract Background and objectivesEvidence of mediastinal Lymph Node Enlargement (LNE) on CT scan is a common finding in idiopathic pulmonary fibrosis (IPF). We sought to investigate whether the involvement of mediastinal lymph nodes is associated with accelerated disease progression, and explored the changes occurring in mediastinal lymph nodes during the radiological follow up of these patients.MethodsThis retrospective study included IPF patients referred to a single ILD centre in Italy. A consensus-based assessment of mediastinal LNE on chest CT scan was performed by two thoracic radiologists. Kaplan-Meier curves and multivariate Cox proportional hazards regression were used to assess hazard ratios for mortality and disease progression (defined as categorical FVC decline ≥10%). The annualized rates of change in functional parameters for each patient were calculated using mixed linear models.Results152 IPF patients were included in the analysis, of whom 135 (89%) received antifibrotic treatment for IPF during the study follow up. Patients having evidence of 3 or more enlarged mediastinal lymph nodes on baseline CT scan showed increased rates of mortality (HR 5.03, 95% CI 1.86-13.62, p≤0.001) and significant disease progression (HR 2.99, 95% CI 1.22-7.33, p=0.17) as compared to patients without LNE, after adjusting for GAP stage. Among 62 patients with LNE who underwent a follow up CT scan of the chest and received antifibrotic treatment, 57 (92%) maintained evidence mediastinal LNE over time.ConclusionsDiffuse mediastinal lymph node involvement predicts clinically meaningful functional deterioration in patients with IPF.

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Impact of mediastinal lymph node enlargement on the prognosis of idiopathic pulmonary fibrosis

PLoS ONE ◽

10.1371/journal.pone.0201154 ◽

2018 ◽

Vol 13 (7) ◽

pp. e0201154 ◽

Cited By ~ 3

Author(s):

Sooim Sin ◽

Kyung Hee Lee ◽

Jee Hye Hur ◽

Sang-hoon Lee ◽

Yeon Joo Lee ◽

...

Keyword(s):

Lymph Node ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Mediastinal Lymph Node ◽

Lymph Node Enlargement

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Mediastinal lymph node enlargement in idiopathic pulmonary fibrosis: relationships with disease progression and pulmonary function trends

10.21203/rs.3.rs-46826/v1 ◽

2020 ◽

Author(s):

Giacomo Sgalla ◽

Anna Rita Larici ◽

Nicoletta Golfi ◽

Mariarosaria Calvello ◽

Alessandra Farchione ◽

...

Keyword(s):

Lymph Node ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Lymph Nodes ◽

Ct Scan ◽

Disease Progression ◽

Mediastinal Lymph Node ◽

Mediastinal Lymph Nodes ◽

Lymph Node Enlargement

Abstract Background and objectives: Evidence of mediastinal Lymph Node Enlargement (LNE) on CT scan is a common finding in idiopathic pulmonary fibrosis (IPF). We sought to investigate whether the involvement of mediastinal lymph nodes is associated with accelerated disease progression, and explored the changes occurring in mediastinal lymph nodes during the radiological follow up of these patients. Methods: This retrospective study included IPF patients referred to a single ILD centre in Italy. A consensus-based assessment of mediastinal LNE on chest CT scan was performed by two thoracic radiologists. Kaplan-Meier curves and multivariate Cox proportional hazards regression were used to assess hazard ratios for mortality and disease progression (defined as categorical FVC decline ≥10%). The annualized rates of change in functional parameters for each patient were calculated using mixed linear models. Results: 152 IPF patients were included in the analysis, of whom 135 (89%) received antifibrotic treatment for IPF during the study follow up. Patients having evidence of 3 or more enlarged mediastinal lymph nodes on baseline CT scan showed increased rates of mortality (HR 5.03, 95% CI 1.86-13.62, p≤0.001) and significant disease progression (HR 2.99, 95% CI 1.22-7.33, p=0.17) as compared to patients without LNE, after adjusting for GAP stage. Among 62 patients with LNE who underwent a follow up CT scan of the chest and received antifibrotic treatment, 57 (92%) maintained evidence mediastinal LNE over time. Conclusions: Diffuse mediastinal lymph node involvement predicts clinically meaningful functional deterioration in patients with IPF.

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Mediastinal lymph node enlargement in idiopathic pulmonary fibrosis: relationships with disease progression and pulmonary function trends

10.21203/rs.3.rs-46826/v3 ◽

2020 ◽

Author(s):

Giacomo Sgalla ◽

Anna Rita Larici ◽

Nicoletta Golfi ◽

Mariarosaria Calvello ◽

Alessandra Farchione ◽

...

Keyword(s):

Lymph Node ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Lymph Nodes ◽

Ct Scan ◽

Disease Progression ◽

Mediastinal Lymph Node ◽

Mediastinal Lymph Nodes ◽

Lymph Node Enlargement

Abstract Background and objectives Evidence of mediastinal Lymph Node Enlargement (LNE) on CT scan is a common finding in idiopathic pulmonary fibrosis (IPF). We sought to investigate whether the involvement of mediastinal lymph nodes is associated with accelerated disease progression, and explored the changes occurring in mediastinal lymph nodes during the radiological follow up of these patients. Methods This retrospective study included IPF patients referred to a single ILD centre in Italy. A consensus-based assessment of mediastinal LNE on chest CT scan was performed by two thoracic radiologists. Kaplan-Meier curves and multivariate Cox proportional hazards regression were used to assess hazard ratios for mortality and disease progression (defined as categorical FVC decline ≥10%). The annualized rates of change in functional parameters for each patient were calculated using mixed linear models. Results 152 IPF patients were included in the analysis, of whom 135 (89%) received antifibrotic treatment for IPF during the study follow up. Patients having evidence of 3 or more enlarged mediastinal lymph nodes on baseline CT scan showed increased rates of mortality (HR 5.03, 95% CI 1.86-13.62, p≤0.001) and significant disease progression (HR 2.99, 95% CI 1.22-7.33, p=0.17) as compared to patients without LNE, after adjusting for GAP stage. Among 62 patients with LNE who underwent a follow up CT scan of the chest and received antifibrotic treatment, 57 (92%) maintained evidence mediastinal LNE over time. Conclusions Diffuse mediastinal lymph node involvement predicts clinically meaningful functional deterioration in patients with IPF.

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Adherence, Persistence, and Effectiveness in Real Life. Multicenter Long-Term Study on the Use of Pirfenidone and Nintedanib in the Treatment of Idiopathic Pulmonary Fibrosis

Journal of Pharmacy Practice ◽

10.1177/08971900211008625 ◽

2021 ◽

pp. 089719002110086

Author(s):

Fiorenzo Santoleri ◽

Luigia Auriemma ◽

Antonella Spacone ◽

Stefano Marinari ◽

Fabio Esposito ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Pharmacological Study ◽

Functional Decline ◽

Real Life ◽

Point Of View ◽

Response To Treatment ◽

First Year ◽

Long Term Study ◽

Second Year

Background: In the treatment of idiopathic pulmonary fibrosis (IPF), nintedanib and pirfenidone, with their different mechanisms of action, lead to a reduction in the rate of progression of the fibrosis process measured by the reduction of functional decline, and, in particular, the decrease in forced vital capacity (FVC) and of the diffusion capacity of the lungs for carbon monoxide (DLCO). The objective of this study was to analyze real-life adherence, persistence and efficacy in the use of pirfenidone and nintedanib in the treatment of IPF. Methods: A non-interventional multicenter retrospective observational pharmacological study in real-life treat-ment at 1 and 2 years was conducted. Furthermore, we analyzed the levels of FVC and DLCO at 6 and 12 months, respectively, from the start of treatment. Results: We identified 144 patients in the period between January 2013 and April 2019. From the point of view of adherence, there is no difference between the two drugs, even though patients who used pirfenidone had increasingly higher values: 0.90 vs 0.89, in the first year, and 0.91 vs 0.84, in the second year. In the first year of treatment, the percentage of persistent patients was 67% and 76%, while in the second year, it dropped to 47% and 53% for pirfenidone and nintedanib, respectively. Conclusion: The stratification of the adherence values as a function of the response to treatment in terms of FVC at 12 months for both study drugs showed that patients with optimal response scored adherence of more than 90%.

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Diagnostic Yield of eXcelon, a New Transbronchial Histology Aspiration Needle in Patients With Mediastinal Lymph Node Enlargement

Journal of Bronchology ◽

10.1097/lbr.0b013e31803815c5 ◽

2007 ◽

Vol 14 (2) ◽

pp. 86-89 ◽

Cited By ~ 3

Author(s):

Frank H. W. Hermens ◽

Frederik B. J. M. Thunnissen ◽

Julius P. Janssen

Keyword(s):

Lymph Node ◽

Diagnostic Yield ◽

Mediastinal Lymph Node ◽

Lymph Node Enlargement

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Prognostic value of mediastinal lymph node enlargement in chronic interstitial lung disease

Diagnostic and Interventional Radiology ◽

10.5152/dir.2021.19585 ◽

2021 ◽

Author(s):

Stefano Grecuccio ◽

◽

Nicola Sverzellati ◽

Elisabetta Uslenghi ◽

Antonella Caminati ◽

...

Keyword(s):

Lymph Node ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Prognostic Value ◽

Mediastinal Lymph Node ◽

Lymph Node Enlargement

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CA 19-9 serum levels in patients with end-stage idiopathic pulmonary fibrosis (IPF) and other interstitial lung diseases (ILDs): Correlation with functional decline

Chronic Respiratory Disease ◽

10.1177/1479973120958428 ◽

2020 ◽

Vol 17 ◽

pp. 147997312095842

Author(s):

Elisabetta Balestro ◽

Gioele Castelli ◽

Nicol Bernardinello ◽

Elisabetta Cocconcelli ◽

Davide Biondini ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Lung Diseases ◽

Lung Transplant ◽

Functional Decline ◽

Serum Levels ◽

Interstitial Lung Diseases ◽

Disease Course ◽

Rapid Progressors ◽

End Stage

Idiopathic pulmonary fibrosis presents a progressive and heterogeneous functional decline. CA 19-9 has been proposed as biomarker to predict disease course, but its role remains unclear. We assessed CA 19-9 levels and clinical data in end-stage ILD patients (48 IPF and 20 non-IPF ILD) evaluated for lung transplant, to correlate these levels with functional decline. Patients were categorized based on their rate of functional decline as slow (n = 20; ΔFVC%pred ≤ 10%/year) or rapid progressors (n = 28; ΔFVC%pred ≥ 10%/year). Nearly half of the entire patients (n = 32; 47%) had CA 19-9 levels ≥37kU/L. CA 19-9 levels in IPF were not different from non-IPF ILD populations, however, the latter group had a median CA 19-9 level above the normal cut-off value of 37 KU/l (60 [17–247] kU/L). Among IPF patients, CA 19-9 was higher in slow than in rapid progressors with a trend toward significance (33vs17kU/L; p = 0.055). In the whole population, CA19-9 levels were inversely related with ΔFVC/year (r = −0.261; p = 0.03), this correlation remained in IPF patients, particularly in rapid progressors (r = −0.51; p = 0.005), but not in non. Moreover, IPF rapid progressors with normal CA 19-9 levels showed the greater ΔFVC/year compared to those with abnormal CA 19-9 (0.95 vs. 0.65 L/year; p = 0.03). In patients with end-stage ILD, CA 19-9 may represent a marker of disease severity, whereas its level is inversely correlated with functional decline, particularly among IPF rapid progressors.

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