An autopsy study of combined pulmonary fibrosis and emphysema: correlations among clinical, radiological, and pathological features

Abstract

BackgroundClinical evaluation to differentiate the characteristic features of pulmonary fibrosis and emphysema is often difficult in patients with combined pulmonary fibrosis and emphysema (CPFE), but diagnosis of pulmonary fibrosis is important for evaluating treatment options and the risk of acute exacerbation of interstitial pneumonia of such patients. As far as we know, it is the first report describing a correlation among clinical, radiological, and whole-lung pathological features in an autopsy cases of CPFE patients.MethodsExperts retrospectively reviewed the clinical charts and examined chest computed tomography (CT) images and pathological findings of an autopsy series of 22 CPFE patients, and compared these with findings from 8 idiopathic pulmonary fibrosis (IPF) patients and 17 emphysema-alone patients.ResultsAll patients had a history of heavy smoking. Forced expiratory volume in 1 s/forced vital capacity (FEV1/FVC%) was significantly lower in the emphysema-alone group than the CPFE and IPF-alone groups. The percent predicted diffusing capacity of the lung for carbon monoxide (DLCO%) was significantly lower in the CPFE group than the IPF- and emphysema-alone groups. Usual interstitial pneumonia (UIP) pattern was observed radiologically in 15 (68.2%) CPFE and 8 (100%) IPF-alone patients and was pathologically observed in all patients from both groups. Pathologically thick-cystic lesions involving one or more acini with dense wall fibrosis and occasional fibroblastic foci surrounded by honeycombing and normal alveoli were confirmed by post-mortem observation as thick-walled cystic lesions (TWCLs). Emphysematous destruction and enlargement of membranous and respiratory bronchioles with fibrosis were observed in the TWCLs. The cystic lesions were always larger than the cysts of honeycombing. The prevalence of both radiological and pathological TWCLs was 72.7% among CPFE patients, but no such lesions were observed in patients with IPF or emphysema alone (p = 0.001). The extent of emphysema in CPFE patients with TWCLs was greater than that in patients without such lesions. Honeycombing with emphysema was also observed in 11 CPFE patients.ConclusionsTWCLs were only observed in the CPFE patients. They were classified as lesions with coexistent fibrosing interstitial pneumonia and emphysema, and should be considered an important pathological and radiological feature of CPFE.

The Syndrome of Combined Pulmonary Fibrosis and Emphysema

“…22.8 ), NSIP, desquamative interstitial pneumonia (with extensive fi brosis), respiratory bronchiolitis -associated ILD [ 7 , 19 , 111 -113 , 122 ]. In an autopsy series of 22 cases including 15 (68 %) with a UIP pattern at HRCT -and 19 with lung cancer -, a pathological pattern of UIP was observed in all cases [ 108 ]. Honeycombing coexisting with emphysema was present in half of the patients.…”

“…They may be associated or not with typical honeycombing, from which they differ by the larger size of the cysts and their often being not clustered, whereas honeycombing corresponds to clustered, cystic air spaces, 3-10 mm and up to 2.5 cm in diameter [ 107 ]. They may be associated with more extensive emphysema at imaging [ 108 ]. Thick-walled large cysts likely result from the development of pulmonary fi brosis in the setting of emphysematous lung, with enlargement of the cysts due to retraction forces in fi brotic lung [ 99 , 109 ].…”

“…Thick-walled large cysts likely result from the development of pulmonary fi brosis in the setting of emphysematous lung, with enlargement of the cysts due to retraction forces in fi brotic lung [ 99 , 109 ]. Enlargement over time of thick-walled large cysts has been described [ 108 ]. We consider that they are one typical feature of the CPFE syndrome [ 21 ].…”

Clinical Impact of Emphysema Evaluated by High-Resolution Computed Tomography on Idiopathic Pulmonary Fibrosis Diagnosed by Surgical Lung Biopsy

“…Not all IIPs with emphysema can be diagnosed by SLB in real life practice, and the clinical features might be different between SLB cases and clinically diagnosed cases. Third, we did not study the pathological features of IPF with emphysema as recently reported by Inomata et al [28]. Fourth, we did not differentiate between centrilobular and paraseptal emphysema.…”

Idiopathic Pulmonary Fibrosis and Emphysema: Between Scylla and Charybdis

“…In the latter group, interobserver agreement on scoring can be poor even amongst experienced thoracic radiologists. The distinction between emphysema and fibrosis on high-resolution computed tomography is expected to be problematic as these two entities are overlapping even on pathology level [20]. Finally, the type of emphysema (paraseptal vs. centrilobular) was not studied.…”