Prognosis of patients with acute exacerbation of combined pulmonary fibrosis and emphysema: a retrospective single-centre study

Ikuyama, Yuichi; Ushiki, Atsuhito; Kosaka, Makoto; Akahane, Jumpei; Mukai, Yuichi; Araki, Taisuke; Kitaguchi, Yoshiaki; Tateishi, Kazunari; Urushihata, Kazuhisa; Matsumura, Yasuo; Yamamoto, Hiroshi; Hanaoka, Masayuki

doi:10.1186/s12890-020-01185-9

“…Previous studies have reported that the prognosis of acute exacerbation is favorable, and that acute exacerbation is less frequent in patients with CPFE than in patients with IPF. However, it may have lethal consequences and careful monitoring is needed [ 73 , 74 ]. Zantah et al [ 74 ] stratified exacerbation events in patients with CPFE into acute exacerbation of COPD (AE-COPD) and AE-IPF.…”

Section: Prognosismentioning

confidence: 99%

Chronic Obstructive Pulmonary Disease Combined with Interstitial Lung Disease

Choi

¹

,

Song

²

,

Rhee

³

2022

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Although chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD) have distinct clinical features, both diseases may coexist in a patient because they share similar risk factors such as smoking, male sex, and old age. Patients with both emphysema in upper lung fields and diffuse ILD are diagnosed with combined pulmonary fibrosis and emphysema (CPFE), which causes substantial clinical deterioration. Patients with CPFE have higher mortality compared with patients who have COPD alone, but results have been inconclusive compared with patients who have idiopathic pulmonary fibrosis (IPF). Poor prognostic factors for CPFE include exacerbation, lung cancer, and pulmonary hypertension. The presence of interstitial lung abnormalities, which may be an early or mild form of ILD, is notable among patients with COPD, and is associated with poor prognosis. Various theories have been proposed regarding the pathophysiology of CPFE. Biomarker analyses have implied that this pathophysiology may be more closely associated with IPF development, rather than COPD or emphysema. Patients with CPFE should be advised to quit smoking and undergo routine lung function tests, and pulmonary rehabilitation may be helpful. Various pharmacologic agents and surgical approaches may be beneficial in patients with CPFE, but further studies are needed.

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“…Previous studies have reported that the prognosis of acute exacerbation is favorable, and that acute exacerbation is less frequent in patients with CPFE than in patients with IPF. However, it may have lethal consequences and careful monitoring is needed 73,74 . Zantah et al stratified exacerbation events in patients with CPFE into acute exacerbation of COPD (AE-COPD) and acute exacerbation of IPF (AE-IPF) 74 .…”

Section: Prognosis Of Cpfementioning

confidence: 99%

Chronic obstructive pulmonary disease combined with interstitial lung disease

Choi¹,

Song²,

Rhee³

2022

Tuberc Respir Dis

3

0

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Although chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD) have distinct clinical features, both diseases may coexist in a patient because they share similar risk factors such as smoking, male sex, and old age. Patients with both emphysema in upper lung fields and diffuse ILD are diagnosed with combined pulmonary fibrosis and emphysema (CPFE), which causes substantial clinical deterioration. Patients with CPFE have higher mortality compared with patients who have COPD alone, but results have been inconclusive compared with patients who have idiopathic pulmonary fibrosis (IPF). Poor prognostic factors for CPFE include exacerbation, lung cancer, and pulmonary hypertension.The presence of interstitial lung abnormalities, which may be an early or mild form of ILD, is notable among patients with COPD, and is associated with poor prognosis. Various theories have been proposed regarding the pathophysiology of CPFE. Biomarker analyses have implied that this pathophysiology may be more closely associated with IPF development, rather than COPD or emphysema. Patients with CPFE should be advised to quit smoking and undergo routine lung function tests, and pulmonary rehabilitation may be helpful. Various pharmacologic agents and surgical approaches may be beneficial in patients with CPFE, but further studies are needed.Additional comparisons of biomarkers between groups revealed four clear categories: biomarkers elevated in emphysema alone (interleukin [IL]-6, CXCL10, CCL2, IL-10, and interferon-γ); biomarkers reduced in emphysema alone (IL-17A, IL-13, matrix metalloproteinase [MMP] 1, MMP13, MMP2, CCL24, and lysyl oxidase); biomarkers that were similar among emphysema, IPF, and CPFE, but different from the healthy control group (sCD14, CCL5, tumor necrosis factor [TNF] RII, SP-D, ST2, IL-1β, IL-4, IL-5, CXCL8, and TNFα); and biomarkers with a mixed pattern that did not fit any other categories. These results suggest that the levels of inflammatory proteins are generally comparable between IPF and CPFE, but distinct features are present in emphysema. These results were also consistent with the findings of previous studies [38][39][40][41] . Therefore, the immunological pathogenesis of CPFE may be more closely associated with IPF development.Klebs von den Lungen-6 (KL-6) is a high-molecular-weight glycoprotein that is strongly expressed on type II alveolar pneumocytes and bronchiolar epithelial cells. It is an important marker of ILD and is associated with disease severity, progression, and survival; notably, it promotes pulmonary fibroblast migration and proliferation [42][43][44][45] . Because of this association with ILD, some studies have investigated relationships with fibrotic changes in patients with COPD. In asthma-COPD overlap, patients with a history of smoking and older age have more frequent ILA 46 . These patients with asthma-COPD overlap and ILA showed higher levels of KL-6, compared with patients with asthma-COPD overlap who lacked ILA.The clinical significance of KL-6 has also bee...

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“…The exact annual risk of AE-CPFE is unknown but appears to be less frequent than AE-IPF. A retrospective study that compared the clinical outcome of patients with AE-CPFE with that of patients with AE-IPF showed a significantly better prognosis and a higher survival rate in AE-CPFE [117]. The treatment and management of patients with AE-CPFE reflect the type of underlying exacerbation (COPD-type or IPF-type).…”

Section: Exacerbations Of Cpfementioning

confidence: 99%

COPD, Pulmonary Fibrosis and ILAs in Aging Smokers: The Paradox of Striking Different Responses to the Major Risk Factors

Beghè

¹

,

Cerri

²

,

Fabbri

³

et al. 2021

IJMS

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Aging and smoking are associated with the progressive development of three main pulmonary diseases: chronic obstructive pulmonary disease (COPD), interstitial lung abnormalities (ILAs), and idiopathic pulmonary fibrosis (IPF). All three manifest mainly after the age of 60 years, but with different natural histories and prevalence: COPD prevalence increases with age to >40%, ILA prevalence is 8%, and IPF, a rare disease, is 0.0005–0.002%. While COPD and ILAs may be associated with gradual progression and mortality, the natural history of IPF remains obscure, with a worse prognosis and life expectancy of 2–5 years from diagnosis. Acute exacerbations are significant events in both COPD and IPF, with a much worse prognosis in IPF. This perspective discusses the paradox of the striking pathological and pathophysiologic responses on the background of the same main risk factors, aging and smoking, suggesting two distinct pathophysiologic processes for COPD and ILAs on one side and IPF on the other side. Pathologically, COPD is characterized by small airways fibrosis and remodeling, with the destruction of the lung parenchyma. By contrast, IPF almost exclusively affects the lung parenchyma and interstitium. ILAs are a heterogenous group of diseases, a minority of which present with the alveolar and interstitial abnormalities of interstitial lung disease.

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Prognosis of patients with acute exacerbation of combined pulmonary fibrosis and emphysema: a retrospective single-centre study

Cited by 10 publications

References 18 publications

Chronic Obstructive Pulmonary Disease Combined with Interstitial Lung Disease

Chronic Obstructive Pulmonary Disease Combined with Interstitial Lung Disease

Chronic obstructive pulmonary disease combined with interstitial lung disease

COPD, Pulmonary Fibrosis and ILAs in Aging Smokers: The Paradox of Striking Different Responses to the Major Risk Factors

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