Clinical Features and Outcomes in Combined Pulmonary Fibrosis and Emphysema in Idiopathic Pulmonary Fibrosis

Ryerson, Christopher J.; Hartman, Thomas E.; Elicker, Brett M.; Ley, Brett; Lee, Joyce; Abbritti, Marta; Jones, Kirk D.; King, Talmadge E.; Ryu, Jay H.; Collard, Harold R.

doi:10.1378/chest.12-2403

Cited by 250 publications

(266 citation statements)

References 22 publications

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“…[8][9][10][11] Our results showed that the CPFE group revealed a significantly worse prognosis than did the non-CPFE group when the threshold of ratio of emphysematous area on HRCT was set at 25%, but no significant difference was found at 10% (Figure 1). Regarding PFTs, a large cohort study of patients with IPF suggested that a decline in FVC of more than 10% in IPF 13 and that in FEV 1 of more than 10% in moderate-to-severe CPFE 28 during a 6-month observation are the best physiological predictors of mortality.…”

Section: Discussionmentioning

confidence: 95%

“…In general, clinical features of CPFE are characterized as follows: 1) % FVC and FEV 1 /FVC are preserved, 2) DLCO is markedly decreased and 3) CPFE is complicated with PH and lung cancer. 9,[23][24][25] However, it was previously clear whether these features hold true when patients are restricted to those with IPF. Our study showed that FVC values were preserved in both the CPFE and the non-CPFE groups, while diffusing capacity (including % DLCO and % KCO) were significantly lower in the CPFE than the non-CPFE group.…”

Section: Discussionmentioning

confidence: 99%

“…8,9,[28][29][30][31] There seem to be two explanations for this: differences in the assessment of emphysema area on HRCT and race differences. Standardization of diagnosis criteria and a large-scale study that takes race into consideration are areas for future research.…”

Section: Discussionmentioning

confidence: 99%

“…Past reports, in which CPFE was defined as having an emphysematous area of 10% or more of the whole lung, showed uncertain prognoses [8][9][10] ; another report suggested that the prognosis of CPFE was poor when it was defined as having an emphysematous area of 25% or greater. 11 Therefore, we considered it necessary to define CPFE based on the emphysematous area reflecting prognosis.…”

Section: Introductionmentioning

confidence: 99%

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Significance of Combined Emphysema in Idiopathic Pulmonary Fibrosis and Serum Surfactant Protein-D as a Prognostic Factor

Yokoo¹,

Shiratori²,

Ikeda³

et al. 2017

Pulm Res Respir Med Open J

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Section: Discussionmentioning

confidence: 95%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Significance of Combined Emphysema in Idiopathic Pulmonary Fibrosis and Serum Surfactant Protein-D as a Prognostic Factor

Yokoo¹,

Shiratori²,

Ikeda³

et al. 2017

Pulm Res Respir Med Open J

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“…La prevalenza di enfisema alla diagnosi oscilla tra il 30% e il 47% e questi pazienti sembrano avere una prognosi peggiore rispetto a quelli che hanno solo IPF. La prognosi è certamente peggiore se si associa anche ipertensione polmonare [24,[47][48][49][50][51]. Dati disponibili di prevalenza di Ipertensione Polmonare Precapillare (PH) in corso di IPF variano dal 31% all'85% [26,[52][53][54][55][56].…”

Section: Comorbiditàunclassified

[Nintedanib for the treatment of idiopathic pulmonary fibrosis in Italy]

Belisari¹,

Bettoni

Cortesi

et al. 2017

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To date, there are few therapeutic answers for Idiopathic pulmonary fibrosis (IPF) and only two pharmacological treatments have a marketing authorization for this disease. Recently nintedanib (Ofev®) has been authorized as a new therapeutic option and its economic profile has been evaluated by international Health Technology Assessment (HTA) bodies. IPF has important implications for everyday life of patients and their carers, negatively influencing their quality of life and bringing heavy economic burden to the NHS and to the entire society. It is, therefore important to consider these aspects for the Italian environment and to perform a pharmacoeconomic evaluation to define the efficiency of nintedanib in IPF by means of a Cost-Utility Analysis (CUA). As IPF is a chronic and progressive disease, a lifetime Markov model has been therefore developed with health states describing the patient’s condition as a combination of lung function and exacerbation history. The cohort entered in the model at different Forced Vital Capacity (FVC%) predicted health states, without exacerbation. The Clinical data used to perform this CUA were derived from clinical trials and the relative efficacy of nintedanib versus the comparator (pirfenidone) was then obtained from a Network Meta-Analysis (NMA) combining data reported in each primary study (INPULSIS 1-2 and TOMORROW trials for nintedanib, and CAPACITY and ASCEND trials for pirfenidone, respectively). At base-case, treatment with nintedanib resulted in a slightly lower estimated total cost vs pirfenidone, better safety profile and lower risk of acute exacerbations with an advantage in Quality Adjusted Life years (QALYs) gained. These results were confirmed by the sensitivity analysis. Although nintedanib appears to be a valuable option for the NHS to treat IPF patients, future data evidence, as long-term or real-life data, will help to confirm these results.[In Italian]

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Pathogenesis, clinical features, and phenotypes of pulmonary hypertension associated with interstitial lung disease: A consensus statement from the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative ‐ Group 3 Pulmonary Hypertension

et al. 2023

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Pulmonary hypertension (PH) is a frequent complication of interstitial lung disease (ILD). Although PH has mostly been described in idiopathic pulmonary fibrosis, it can manifest in association with many other forms of ILD. Associated pathogenetic mechanisms are complex and incompletely understood but there is evidence of disruption of molecular and genetic pathways, with panvascular histopathologic changes, multiple pathophysiologic sequelae, and profound clinical ramifications. While there are some recognized clinical phenotypes such as combined pulmonary fibrosis and emphysema and some possible phenotypes such as connective tissue disease associated with ILD and PH, the identification of further phenotypes of PH in ILD has thus far proven elusive. This statement reviews the current evidence on the pathogenesis, recognized patterns, and useful diagnostic tools to detect phenotypes of PH in ILD. Distinct phenotypes warrant recognition if they are characterized through either a distinct presentation, clinical course, or treatment response. Furthermore, we propose a set of recommendations for future studies that might enable the recognition of new phenotypes.

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Clinical Features and Outcomes in Combined Pulmonary Fibrosis and Emphysema in Idiopathic Pulmonary Fibrosis

Cited by 250 publications

References 22 publications

Significance of Combined Emphysema in Idiopathic Pulmonary Fibrosis and Serum Surfactant Protein-D as a Prognostic Factor

Significance of Combined Emphysema in Idiopathic Pulmonary Fibrosis and Serum Surfactant Protein-D as a Prognostic Factor

[Nintedanib for the treatment of idiopathic pulmonary fibrosis in Italy]

Pathogenesis, clinical features, and phenotypes of pulmonary hypertension associated with interstitial lung disease: A consensus statement from the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative ‐ Group 3 Pulmonary Hypertension

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