Combined cryptogenic fibrosing alveolitis andemphysema: the value of high resolution computed tomography in assessment

Wiggins, J; Strickland, Basil; Turner-Warwick, M

doi:10.1016/s0954-6111(08)80070-4

Cited by 210 publications

(160 citation statements)

References 17 publications

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“…5,[27][28][29][30][31] Currently, it is unclear whether there is any discrete pathobiological mechanism responsible for the natural history of CPFE, or are there merely co-existing fibrotic (eg, UIP) and emphysematous lesions present in those exposed to the shared environmental risk factor, smoke. 32,33 A recent hypothesis suggests that fibrosis and emphysema share common pathogenetic mechanisms of accelerated senescence via telomere length abnormalities.…”

Section: Discussionmentioning

confidence: 99%

Idiopathic Pulmonary Fibrosis With Emphysema: Evidence of Synergy Among Emphysema and Idiopathic Pulmonary Fibrosis in Smokers

et al. 2014

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BACKGROUND: Emphysema and fibrosis, typically the idiopathic pulmonary fibrosis (IPF) form of usual interstitial pneumonia (UIP), can co-exist as combined pulmonary fibrosis emphysema (CPFE). It is unknown whether there is a pathobiologic basis for CPFE beyond the coexistence of fibrosis and emphysema. The aim of this study was to ascertain radiologic differences in severity of fibrosis and emphysema in smokers with IPF versus other forms of UIP. METHODS: Computed tomography thorax images were prospectively rescored in retrospectively identified smokers (minimum 5-pack-year history) with radiologic UIP (any etiology). Radiologic severity (emphysema/fibrosis/reticulation) was scored in consensus by two radiologists, blinded to clinical details, across 5 lung regional levels, and then correlated with clinical data. RESULTS: For the whole cohort (IPF, n ‫؍‬ 102; non-IPF UIP [mainly rheumatoid arthritis/asbestosis/scleroderma], n ‫؍‬ 30), IPF and non-IPF UIP smokers were similar regarding pack-year, age, gender, and lung function (P > .1). IPF smokers had greater whole lung fibrosis and reticulation scores (P < .04 in all cases). CPFE was present in n ‫؍‬ 61 (IPF, n ‫؍‬ 49; non-IPF UIP, n ‫؍‬ 12). Compared with smokers with non-IPF CPFE, smokers with IPF and emphysema (IPFE) were similar regarding confounders (P > .1). There were significantly greater regional reticulation severity (P ‫؍‬ .009), cumulative emphysema severity (P ‫؍‬ .04), and cumulative reticulation severity (P < .001) scores in IPFE versus non-IPF CPFE. CONCLUSIONS: When controlled for confounders, smokers with IPFE have worse radiologic CPFE than other smokers with non-IPF UIP and emphysema, suggesting an interactive synergy among IPF, emphysema, and smoking, with more extensive emphysema due to either inherent susceptibility and/or traction effects. IPFE should be considered separately from other CPFE in future work. It is currently unknown whether CPFE is a distinct pathobiologic entity; therefore, we identified subjects with radiologic UIP (any etiology) who had been similarly exposed to smoke, and asked whether there are differences in the extent/severity of radiologic fibrosis and/or emphysema in those with IPF versus individuals with non-IPF UIP. Although relevant confounders were similar, IPF smokers had greater whole lung fibrosis and reticulation scores than smokers with secondary forms of UIP, and in the CPFE subgroup, smokers with IPF/emphysema had worse radiologic CPFE findings than smokers with non-IPF UIP/emphysema. It is shown for the first time that relevant confounding variables do not explain the observed excess radiologic severity of emphysema and fibrosis in smokers with IPF compared with smokers with non-IPF UIP, lending support to the hypothesis that there is a pathobiologic mechanism or synergy involved in IPF with emphysema that is distinct from the mere co-existence of UIP and emphysematous processes.

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Section: Discussionmentioning

confidence: 99%

Idiopathic Pulmonary Fibrosis With Emphysema: Evidence of Synergy Among Emphysema and Idiopathic Pulmonary Fibrosis in Smokers

et al. 2014

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“…The syndrome of CPFE results from the association of distinct features and symptoms, that include severe dyspnoea, unexpected subnormal spirometry findings, severely impaired transfer capacity for carbon monoxide, hypoxaemia at exercise, and characteristic imaging features [8,[13][14][15]. Basal crackles are heard on auscultation (table 1) [4,9].…”

Section: Disease Characteristicsmentioning

confidence: 99%

The impact of emphysema in pulmonary fibrosis

Cottin¹

2013

European Respiratory Review

130

115

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Several groups have described a syndrome in which idiopathic pulmonary fibrosis (IPF) coexists with pulmonary emphysema. This comes as no surprise since both diseases are associated with a history of exposure to cigarette smoke. The syndrome of combined pulmonary fibrosis and emphysema (CPFE) is characterised by upper lobe emphysema and lower lobe fibrosis. Physiological testing of these patients reveals preserved lung volume indices contrasted by markedly impaired diffusion capacity. The incidence of CPFE remains unknown but several case series suggest that this subgroup may comprise up to 35% of patients with IPF. CPFE is a strong determinant of associated pulmonary hypertension (PH). In addition, CPFE has major effects on measures of physiological function, exercise capacity and prognosis, and may affect the results of pulmonary fibrosis trials. Further studies are needed to ascertain the aetiology, morbidity, mortality and management of the CPFE syndrome, with or without PH, and to evaluate novel therapeutic options in CPFE.

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“…This coexistence of pulmonary fibrosis and emphysema was first described in 1990 by Wiggins et al 1 The term CPFE was coined by Cottin et al in 2005 as a syndrome of heterogeneous population of patients which is diagnosed by high resolution computed tomography (HRCT). This disease is rare.…”

Section: Introductionmentioning

confidence: 97%