Combined pulmonary fibrosis and emphysema in systemic sclerosis: A syndrome associated with heavy morbidity and mortality

Champtiaux, Nicolas; Cottin, Vincent; Chassagnon, Guillaume; Chaigne, Benjamin; Valeyre, Dominique; Nunès, Hilario; Hachulla, É.; Launay, David; Crestani, Bruno; Cazalets, C.; Jégo, Patrick; Bussone, Guillaume; Bérezné, Alice; Guillevin, Loı̈c; Revel, Marie-Pierre; Cordier, J; Mouthon, Luc

doi:10.1016/j.semarthrit.2018.10.011

Cited by 38 publications

(52 citation statements)

References 32 publications

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“…Many nonIPF fILD cases may present with a combination of emphysema and fibrosis (CPFE). In systemic sclerosis-associated ILD (Ssc-ILD), 7.8% of cases have been shown to present with CPFE [ 25 ], while almost 30% of rheumatoid arthritis-associated ILD (RA-ILD) cases have concomitant emphysema [ 26 ]. Only one-third of CPFE patients are estimated to have IPF [ 27 ].…”

Section: Defining Progressiveness: From Clinical Trial Endpoint Tomentioning

confidence: 99%

Towards the Essence of Progressiveness: Bringing Progressive Fibrosing Interstitial Lung Disease (PF-ILD) to the Next Stage

Sadeleer

Goos

Yserbyt

et al. 2020

JCM

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Although only recently introduced in the ILD community, the concept of progressive fibrosing interstitial lung disease (PF-ILD) has rapidly acquired an important place in the management of non-idiopathic pulmonary fibrosis fibrosing ILD (nonIPF fILD) patients. It confirms a clinical gut feeling that an important subgroup of nonIPF fILD portends a dismal prognosis despite therapeutically addressing the alleged triggering event. Due to several recently published landmark papers showing a treatment benefit with currently available antifibrotic drugs in PF-ILD patients, endorsing a PF-ILD phenotype has vital therapeutic consequences. Importantly, defining progressiveness is based on former progression, which has proven to be a rather moderate predictor of future progression. As fibrosis extent >20% and the presence of honeycombing have superior predictive properties regarding future progression, we advocate immediate initiation of antifibrotic treatment in the presence of these risk factors. In this perspective, we describe the historical context wherein PF-ILD has emerged, determine the currently employed PF-ILD criteria and their inherent limitations and propose new directions to mature its definition. Finally, while ascertaining progression in a nonIPF fILD patient clearly demonstrates the need for (additional) therapy, in the future, therapeutic decisions should be taken after assessing which pathway is ultimately driving the progression. Although not readily available, pathophysiological insight and diagnostic means are emergent to go full steam ahead in this novel direction.

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Section: Defining Progressiveness: From Clinical Trial Endpoint Tomentioning

confidence: 99%

Towards the Essence of Progressiveness: Bringing Progressive Fibrosing Interstitial Lung Disease (PF-ILD) to the Next Stage

Sadeleer

Goos

Yserbyt

et al. 2020

JCM

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show abstract

“…3). 31,32 The presence of emphysema in SSc-ILD may confound screening algorithms for PAH, through its impact on gas transfer measurements. 32 Interestingly, a proportion of patients with SSc-ILD and emphysema are never smokers, 32 suggesting a possible role for inflammatory/fibrotic processes in the pathogenesis of emphysema independently of cigarette smoke.…”

Section: Ild Patterns In Sscmentioning

confidence: 99%

Systemic Sclerosis Associated Interstitial Lung Disease: A Comprehensive Overview

Mackintosh

Stainer

Barnett

et al. 2019

Semin Respir Crit Care Med

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Interstitial lung disease (ILD) is a common complication of systemic sclerosis (SSc). SSc-ILD adversely impacts quality of life and is currently the leading cause of death in this multisystem disease. Identifying clinically significant SSc-ILD is critically important. Accurate staging and prognostication remain difficult; however, significant advances have been made in the last decade. Evidence supports the need to treat patients with extensive and/or progressive SSc-ILD, while only a subset of patients with limited ILD may require treatment. Research is urgently required to allow improved prediction of patients at risk of ILD progression at an early point in the disease, and ideally prior to its onset, to allow prevention. The last decade has seen the publication of landmark clinical trials for SSc-ILD. More effective strategies with less toxicity are under investigation. For those with refractory or very advanced disease, studies into disease-specific palliative approaches are in their infancy. Lung transplantation as an option for SSc-ILD remains patient- and center-specific, with data to suggest equivalent outcomes to other fibrotic lung diseases, in carefully selected cases. This review aims to provide a comprehensive overview of all key aspects of SSc-ILD.

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“…In contrast, CPFE patients are likely to exhibit paraseptal emphysema (30%-65%) [ 28 , 29 ], which is consistent with the present study. CPFE with paraseptal emphysema lesions is associated with CTD and a poor prognosis [ 30 – 32 ]. Considering emphysema subtypes in CPFE might contribute to the putative clinical course.…”

Section: Discussionmentioning

confidence: 99%

Objective quantitative multidetector computed tomography assessments in patients with combined pulmonary fibrosis with emphysema: Relationship with pulmonary function and clinical events

et al. 2020

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Background Combined pulmonary fibrosis with emphysema (CPFE) is a clinically meaningful syndrome characterized by coexisting upper-lobe emphysema and lower-lobe interstitial fibrosis. However, ambiguous diagnostic criteria and, particularly, the absence of objective methods to quantify emphysematous/fibrotic lesions in patients with CPFE confound the interpretation of the pathophysiology of this syndrome. We analyzed the relationship between objectively quantified computed tomography (CT) measurements and the results of pulmonary function testing (PFT) and clinical events in CPFE patients. Materials and methods We enrolled 46 CPFE patients who underwent CT and PFT. The extent of emphysematous lesions was obtained by calculating the percent of low attenuation area (%LAA). The extent of fibrotic lesions was calculated as the percent of high attenuation area (%HAA). %LAA and %HAA values were combined to yield the percent of abnormal area (%AA). We assessed the relationships between CT parameters and other clinical indices, including PFT results. Multivariate analysis was performed to examine the association between the CT parameters and clinical events. Results A greater negative correlation with percent predicted diffusing capacity of the lung for carbon monoxide (DL CO %predicted) existed for %AA (r = -0.73, p < 0.001) than for %LAA or %HAA alone. The %HAA value was inversely correlated with percent predicted forced vital capacity (r = -0.48, p < 0.001), percent predicted total lung capacity (r = -0.48, p < 0.01), and DL CO %predicted (r = -0.47, p < 0.01). Multivariate logistic regression analysis found that %AA showed the strongest association with hospitalization events (odds ratio = 1.20, 95% confidence interval = 1.01–1.54, p = 0.029). Conclusion Quantitative CT measurements reflected deterioration in pulmonary function and were associated with hospitalization in patients with CPFE. This approach could serve as a useful method to determine the extent of lung morphology, pathophysiology, and the clinical course of patients with CPFE.

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Combined pulmonary fibrosis and emphysema in systemic sclerosis: A syndrome associated with heavy morbidity and mortality

Cited by 38 publications

References 32 publications

Towards the Essence of Progressiveness: Bringing Progressive Fibrosing Interstitial Lung Disease (PF-ILD) to the Next Stage

Towards the Essence of Progressiveness: Bringing Progressive Fibrosing Interstitial Lung Disease (PF-ILD) to the Next Stage

Systemic Sclerosis Associated Interstitial Lung Disease: A Comprehensive Overview

Objective quantitative multidetector computed tomography assessments in patients with combined pulmonary fibrosis with emphysema: Relationship with pulmonary function and clinical events

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