Comparison of clinical characteristics and prognostic factors of combined pulmonary fibrosis and emphysema versus idiopathic pulmonary fibrosis alone

Sugino, Keishi; Ishida, Fumiaki; Kikuchi, Naoshi; Hirota, Nao; Sano, Go; Sato, Keita; Isobe, Kazutoshi; Sakamoto, Susumu; Takai, Yujiro; Homma, Sakae

doi:10.1111/resp.12207

Cited by 83 publications

(86 citation statements)

References 21 publications

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“…All patients with CPFE were current or ex-smokers (19). Sugino et al reported that the prevalence of LC among patients with CPFE is higher than that in patients with IPF alone (20). CPFE is also observed in patients with CTD-ILD and may be associated with an increased risk of LC (9).…”

Section: Discussionmentioning

confidence: 99%

Lung cancer in connective tissue disease-associated interstitial lung disease: clinical features and impact on outcomes

Watanabe¹,

Saeki²,

Waseda³

et al. 2018

J. Thorac. Dis.

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Backgrounds: Lung cancer (LC) adversely impacts survival in patients with idiopathic pulmonary fibrosis.However, little is known about LC in patients with connective tissue disease-associated interstitial lung disease (CTD-ILD). The aim of this study was to evaluate the prevalence of and risk factors for LC in CTD-ILD, and the clinical characteristics and survival of CTD-ILD patients with LC. Results: Of 266 patients with CTD-ILD, 24 (9.0%) had LC. CTD-ILD with LC was more likely in patients who were older, male, and smokers; had rheumatoid arthritis, a usual interstitial pneumonia pattern, emphysema on chest computed tomography scan, and lower diffusing capacity of the lung carbon monoxide (DLco)% predicted; and were not receiving immunosuppressive therapy. Multivariate analysis indicated that the presence of emphysema [odds ratio (OR), 8.473; 95% confidence interval (CI), 2.241-32.033] and nonuse of immunosuppressive therapy (OR, 8.111; 95% CI, were independent risk factors for LC. CTD-ILD patients with LC had significantly worse survival than patients without LC (10-year survival rate: 28.5% vs. 81.8%, P<0.001).Conclusions: LC is associated with the presence of emphysema and nonuse of immunosuppressive therapy, and contributes to increased mortality in patients with CTD-ILD.

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Section: Discussionmentioning

confidence: 99%

Lung cancer in connective tissue disease-associated interstitial lung disease: clinical features and impact on outcomes

Watanabe¹,

Saeki²,

Waseda³

et al. 2018

J. Thorac. Dis.

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“…Mejía et al (3) reported that survival of CPFE patients was poorer compared with that of patients with idiopathic pulmonary fibrosis (IPF) alone and that pulmonary hypertension in CPFE patients was an independent predictor of mortality. Previous studies have indicated that CPFE patients have a significantly increased risk of lung cancer compared with patients with chronic obstructive pulmonary disease (COPD) or IPF alone (1,4,5). Post-operative acute exacerbation (AE) of interstitial lung disease in patients with lung cancer is associated with a high mortality rate (43.9%) (6).…”

Section: Introductionmentioning

confidence: 99%

Clinical features and outcomes of patients with lung cancer as well as combined pulmonary fibrosis and emphysema

Otsuka

Sugino

Hata

et al. 2016

Molecular and Clinical Oncology

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Abstract. The syndrome of combined pulmonary fibrosis and emphysema (CPFE) has been characterized by severely impaired gas exchange and poor survival. However, the clinical features of patients with lung cancer plus CPFE have remained elusive. The present study performed a retrospective analysis to examine the clinical characteristics and outcome of surgically resected patients with lung cancer plus CPFE. Among 831 patients with primary lung cancer who underwent surgical resection, 23 patients (2.8%) were diagnosed with CPFE and 9 patients (1.1%) with solely idiopathic pulmonary fibrosis (IPF). Thirty-five patients were stratified as the solely emphysema group with adjustment of the pathological stage. The clinicopathological characteristics of patients in the CPFE group and their outcomes were evaluated and compared with those with the solely IPF or solely emphysema groups. Within the CPFE group, no significant differences in survival between patients with post-operative acute exacerbation (AE; n=3) and those without AE (n=20) were noted; however, in the solely IPF group, patients with post-operative AE (n=4) had a significantly shorter survival than those without AE (n=5; P=0.022). The 5-year survival rate of patients in the CPFE, solely IPF and solely emphysema groups was 22, 22 and 58%, respectively. Furthermore, the CPFE and solely IPF groups showed a significantly shorter survival than the solely emphysema group (P=0.001 and 0.011, respectively). In conclusion, surgically resected lung cancer patients with CPFE had poor survival, which was, in contrast to that of lung cancer patients with solely IPF, not affected by AE. IntroductionThe syndrome of combined pulmonary fibrosis and emphysema (CPFE) has been suggested to be a clinically important phenotype of pulmonary fibrosis (1). Cottin et al (1) defined CPFE as a distinct condition, with characteristic features in the chest computed tomography (CT) image that include emphysema in the upper pulmonary region concomitant with parenchymal fibrosis in the lower region. The syndrome is characterized by sub-normal spirometry, severely impaired gas exchange, high prevalence of pulmonary hypertension and poor survival (2-4). Mejía et al (3) reported that survival of CPFE patients was poorer compared with that of patients with idiopathic pulmonary fibrosis (IPF) alone and that pulmonary hypertension in CPFE patients was an independent predictor of mortality. Previous studies have indicated that CPFE patients have a significantly increased risk of lung cancer compared with patients with chronic obstructive pulmonary disease (COPD) or IPF alone (1,4,5). Post-operative acute exacerbation (AE) of interstitial lung disease in patients with lung cancer is associated with a high mortality rate (43.9%) (6). However, the clinical features and prognosis of patients with lung cancer and CPFE have remained elusive. The present study performed a retrospective analysis to determine the prevalence, clinical characteristics and outcome of patients with surgically resected lung canc...

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“…The prevalence of COPD (including emphysema) was reported in 23 studies (online supplementary table S1), of which eight were from Europe [18][19][20][21][22][23][24][25], eight were from North America [6,[26][27][28][29][30][31][32], six were from Asia [33][34][35][36][37][38] and one was from South America (Brazil) [39]. The reported prevalence of COPD ranged from 6% to 67% (figure 2) ( prevalence ranges by country: 12% [21] to 51% [18] in Europe, 8% [26] to 67% [31] in North America and 34% [36] to 65% [34] in Asia).…”

Section: Respiratory Comorbidities Copdmentioning

confidence: 99%

Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review

et al. 2015

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Idiopathic pulmonary fibrosis (IPF) is associated with a fatal prognosis and manifests in patients over 60 years old who may have comorbidities. The prevalence and impact of comorbidities on the clinical course of IPF is unclear.This systematic literature review examined the prevalence of comorbidities and mortality associated with comorbidities in IPF patients. Relevant observational studies published in English from January 1990 to January 2015 identified via MEDLINE and EMBASE were included; bibliographies of articles were also searched.Among the 126 studies included, prevalence of pulmonary hypertension (PH) was 3-86%, 6-91% for obstructive sleep apnoea, 3-48% for lung cancer and 6-67% for chronic obstructive pulmonary disease (COPD). Nonrespiratory comorbidities included ischaemic heart disease (IHD) (3-68%) and gastrooesophageal reflux (GER) (0-94%). Mortality was highest among patients with IPF and lung cancer. Most studies assessed relatively small samples of patients with IPF.PH, COPD, lung cancer, GER and IHD are significant comorbidities; differences in IPF severity, case definitions and patient characteristics limited the comparability of findings. The identification and prompt treatment of comorbidities may have a clinically significant impact on overall outcome that is meaningful for patients with IPF. @ERSpublications Identification and prompt treatment of comorbidities has a clinically significant impact on outcome for IPF patients

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Comparison of clinical characteristics and prognostic factors of combined pulmonary fibrosis and emphysema versus idiopathic pulmonary fibrosis alone

Abstract: This study demonstrated that the prognosis of CPFE is significantly worse than that of IPF alone. In particular, CPFE with paraseptal emphysema associated with high esPAP has an extremely poor prognosis.

Cited by 83 publications

References 21 publications

Lung cancer in connective tissue disease-associated interstitial lung disease: clinical features and impact on outcomes

Lung cancer in connective tissue disease-associated interstitial lung disease: clinical features and impact on outcomes

Clinical features and outcomes of patients with lung cancer as well as combined pulmonary fibrosis and emphysema

Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review

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