Combined Pulmonary Fibrosis and Emphysema

Bolaki, Maria; Αντωνίου, Κατερίνα

doi:10.1055/s-0040-1708058

Cited by 12 publications

(10 citation statements)

References 59 publications

(52 reference statements)

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“…Cluster 3 had a high prevalence of emphysema. Pulmonary hypertension was not frequent in this cluster, and we did not observe the association between combined pulmonary fibrosis and emphysema and PH that has been described in other studies [ 28 ]. Patients in cluster 1 experienced an annual decline in FVC% predicted, as did patients in cluster 2.…”

Section: Discussioncontrasting

confidence: 70%

Comorbidities in unclassifiable interstitial lung disease

et al. 2022

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Background Comorbidities are common in interstitial lung diseases (ILD) and have an important association with survival, but the frequency and prognostic impact of comorbidities in unclassifiable interstitial lung disease (uILD) remains elusive. We aimed to describe the prevalence of comorbidities and assess the impact on survival in patients with uILD. Furthermore, we aimed to identify and characterize potential phenotypes based on clusters of comorbidities and examine their association with disease progression and survival. Methods Incident patients diagnosed with uILD were identified at two ILD referral centers in Denmark and Germany from 2003 to 2018. The diagnosis uILD was based on multidisciplinary team meetings. Clinical characteristics and comorbidities were extracted from ILD registries and patient case files. Survival analyses were performed using Cox regression analyses, disease progression was analyzed by linear mixed effects models, and clusters of comorbidities were analyzed using self-organizing maps. Results A total of 249 patients with uILD were identified. The cohort was dominated by males (60%), former (49%) or current (15%) smokers, median age was 70 years, mean FVC was 75.9% predicted, and mean DLCO was 49.9% predicted. One-year survival was 89% and three-year survival was 73%. Eighty-five percent of the patients had ≥ 1 comorbidities, 33% had ≥ 3 comorbidities and 9% had ≥ 5 comorbidities. The only comorbidity associated with excess mortality was dyslipidemia. No association between survival and number of comorbidities or the Charlson comorbidity index was observed. Three clusters with different comorbidities profiles and clinical characteristics were identified. A significant annual decline in FVC and DLCO % predicted was observed in cluster 1 and 2, but not in cluster 3. No difference in mortality was observed between the clusters. Conclusions The comorbidity burden in uILD is lower than reported in other types of ILD and the impact of comorbidities on mortality needs further clarification. Three clusters with distinct comorbidity profiles were identified and could represent specific phenotypes. No difference in mortality was observed between clusters, but slower disease progression was observed in cluster 3. Better understanding of disease behavior and mortality will require further studies of subgroups of uILD with longer observation time.

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Section: Discussioncontrasting

confidence: 70%

Comorbidities in unclassifiable interstitial lung disease

et al. 2022

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“…Cluster 3 had a high prevalence of emphysema. Pulmonary hypertension was not frequent in this cluster, and we did not observe the association between combined pulmonary brosis and emphysema and PH that has been described in other studies [24]. Patients in cluster 1 experienced an annual decline in FVC% predicted, as did patients in cluster 2.…”

Section: Discussioncontrasting

confidence: 60%

Comorbidities in Unclassifiable Interstitial Lung Disease

Prior

Hyldgaard

Torrisi³

et al. 2021

Preprint

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BackgroundComorbidities are common in interstitial lung diseases (ILD) and have an important association with survival, but the frequency and prognostic impact of comorbidities in unclassifiable interstitial lung disease (uILD) remains elusive. We aimed to describe the prevalence of comorbidities and assess the impact on survival in patients with uILD. Furthermore, we aimed to identify and characterize potential phenotypes based on clusters of comorbidities and examine their association with disease progression and survival.MethodsIncident patients diagnosed with uILD were identified at two ILD referral centers in Denmark and Germany from 2003 to 2018. The diagnosis uILD was based on multidisciplinary team meetings. Clinical characteristics and comorbidities were extracted from ILD registries and patient case files. Survival analyses were performed using Cox regression analyses, disease progression was analyzed by linear mixed effects models, and clusters of comorbidities were analyzed using self-organizing maps.ResultsA total of 249 patients with uILD were identified. The cohort was dominated by males (60%), former (49%) or current (15%) smokers, median age was 70 years, mean FVC was 75.9% predicted, and mean DLCO was 49.9% predicted. One-year survival was 89% and three-year survival was 73%. Eighty-five percent of the patients had ≥1 comorbidities, 33% had ≥3 comorbidities and 9% had ≥5 comorbidities. The only comorbidity associated with excess mortality was dyslipidemia. No association between survival and number of comorbidities or the Charlson comorbidity index was observed. Three clusters with different comorbidities profiles and clinical characteristics were identified. A significant annual decline in FVC and DLCO % predicted was observed in cluster 1 and 2, but not in cluster 3. No difference in mortality was observed between the clusters.ConclusionsThe comorbidity burden in uILD is lower than in other types of ILD and the impact of comorbidities on mortality is less clear. Three clusters with distinct comorbidity profiles were identified and could represent specific phenotypes. No difference in mortality was observed between clusters, but slower disease progression was observed in cluster 3. Better understanding of disease behavior and mortality will require further studies of subgroups of uILD with longer observation time.

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“…Malli et al analyzed CPFE patients of a Greek cohort, and revealed that ILD extent of more than 30% was associated with poor survival 76 . In contrast, Jacob et al analyzed 272 IPF patients, and reported that presence or extent of emphysematous area in volumetric chest CT image were not associated with patients' survival 82,83 . Thus, revelation of abnormal lesion in CPFE patients is important in predicting the disease prognosis, and use of late gadolinium enhanced thoracic magnetic resonance imaging (LGE-MRI) may have a role in differentiation from pure emphysema 84 .…”

Section: Prognosis Of Cpfementioning

confidence: 97%

Chronic obstructive pulmonary disease combined with interstitial lung disease

Choi¹,

Song²,

Rhee³

2022

Tuberc Respir Dis

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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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Combined Pulmonary Fibrosis and Emphysema

Cited by 12 publications

References 59 publications

Comorbidities in unclassifiable interstitial lung disease

Comorbidities in unclassifiable interstitial lung disease

Comorbidities in Unclassifiable Interstitial Lung Disease

Chronic obstructive pulmonary disease combined with interstitial lung disease

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