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

doi:10.1016/S0954-6111(08)80070-4

Respiratory Medicine

Volume 84, Issue 5, September 1990, Pages 365-369

https://doi.org/10.1016/S0954-6111(08)80070-4 Get rights and content

Simple tests of lung function may be misleading in the assessment of patients with interstitial lung disease. Eightpatients are described with cryptogenic fibrosing alveolitis (histologically proven in four) with severe breathlessness and low gas transfer (median DLCO 32·4%, range 9·2 to 35·3%, % predicted) in whom lung volumes were preserved [median VC 98·7, range 67·5–131·1%; median TLC 92·5, range 88·1 to 121·2, (% predicted)]. and in whom there was no evidence of airflow obstruction [median FEV₁/FVC 84·6, range 68–116 (% predicted)]. All were current or ex-heavy smokers. Thoracic high resolution computed tomography revealed upper zone emphysema, the extent of which was not appreciated using conventional chest radiography. The atypical physiological and radiological features can be explained by coincidental cryptogenic fibrosing alveolitis and emphysema and high resolution computed tomography was valuable in the assessment of these patients.

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Combined pulmonary fibrosis and emphysema (CPFE) syndrome refers to co-occurrence of two disease processes in the lung that can be difficult to diagnose but is associated with high morbidity and mortality burden. Diagnosis of CPFE is challenging because the two diseases can counterbalance respective impairments resulting in deceivingly normal-appearing chest radiography and spirometry in a dyspneic patient. Although an international committee published the terminology and definitions of CPFE in 2022, consensus on exact diagnostic criteria and optimal management strategy is yet to be determined. Herein, we provide a narrative review summarizing the literature on CPFE from 1990 to 2022, including historical background, epidemiology, pathogenesis, clinical features, imaging and pulmonary function findings, diagnosis, prognosis, complications, and treatment. Although CPFE was initially conceived as a variant presentation of idiopathic pulmonary fibrosis, it has been recognized to occur in patients with a wide variety of interstitial lung diseases, including connective tissue disease–associated interstitial lung diseases, and hypersensitivity pneumonitis. The affected patients have a heightened risk for pulmonary hypertension and lung cancer. Clinicians need to recognize the characteristic presenting features of CPFE along with prognostic implications of this entity.
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Combined pulmonary fibrosis and emphysema (CPFE) is a distinct clinical entity that can progress to end-stage lung disease. Patients with CPFE may develop pulmonary hypertension and face a predicted 1-year mortality of 60%. Lung transplantation is the only curative therapeutic option for CPFE. This report describes our experience after lung transplantation in patients with CPFE.
This retrospective, single-center study describes short- and long-term outcomes for adult patients who underwent lung transplant for CPFE.
The study included 19 patients with explant pathology-proven diagnosis of CPFE. The patients were transplanted between July 2005 and December 2018. Sixteen recipients (84%) had pulmonary hypertension before transplant. Of the 19 patients, 7 (37%) had primary graft dysfunction at 72 hours post-transplant. 1-, 3-, and 5-year freedom from bronchiolitis obliterans syndrome was 100%, 91% (95% CI, 75%–100%), and 82% (95% CI, 62%–100%), respectively. One-, 3-, and 5-year survival was 94% (95% CI, 84%–100%), 82% (95% CI, 65%–100%), and 74% (95% CI, 54%–100%), respectively.
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Citation Excerpt :
Original descriptors of combined pulmonary fibrosis and emphysema (CPFE) suggest radiologic upper lobe emphysema and lower lobe lung fibrosis in the same patient. While first formally described by Cottin et al., in 2005(1), an initial report of eight patients presenting with CPFE was published by Wiggins et al., in 1990 [2]. Since then, several single and multicenter cohorts have reviewed clinical findings at presentation, assessment of pulmonary function findings over time [3–5], and predictors of death or outcome(6–9).
The prevalence of classifiable and unclassifiable causes of lung fibrosis and its implications for survival are mostly unknown in combined pulmonary fibrosis and emphysema (CPFE).
Patients with >10% involvement of both emphysema and lung fibrosis seen over 11 years at our institution were reviewed independently by expert radiologists for fibrotic and emphysematous findings and overall fibrotic CT pattern. Underlying interstitial lung disease (ILD) diagnoses and baseline demographic and clinical characteristics were collated and assessed for predictors of comparative survival.
In this retrospective cohort, 179 CPFE patients were identified and categorized as 58 usual interstitial pneumonia/idiopathic pulmonary fibrosis (UIP/IPF) (32%), 42 secondary ILD (23%), and 79 unclassifiable ILD (44%). The most prevalent (47%) radiologic pattern was ‘unclassifiable’, followed by ‘consistent’ and ‘possible’ UIP pattern in 38%. Adjusted predictors of mortality for the cohort as a whole included age (HR 1.03[1.01–1.06], P = 0.002), percent predicted diffusing capacity for carbon monoxide (unit HR 0.97 [0.96–0.99], P = 0.001), honeycombing (HR 1.58 [1.02–2.43], P = 0.04), and right ventricular dysfunction (HR 2.28 [1.39–3.97], P = 0.002). Survival was similar between CPFE with secondary ILD and CPFE with UIP/IPF, while CPFE with unclassifiable ILD had better comparative survival (Log rank = 0.026).
Our findings suggest only about a third of CPFE patients represent suspected UIP/IPF; the majority were clinically and radiologically unclassifiable ILD whose survival was comparatively better. Identifiable or secondary causes of lung fibrosis in CPFE occurred in about a fifth of presenting patients.
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Combined cryptogenic fibrosing alveolitis andemphysema: the value of high resolution computed tomography in assessment

Clin Radiol

Am J Med

Chest

Chest

Chest

Cryptogenic fibrosing alveolitis; clinical features and their influence on survival

Thorax

Idiopathic pulmonary fibrosis. Clinical, histologic, radiographic, physiologic, scintigraphic, cytology and biochemical aspects

An Intern Med

Fibrosing alveolitis: CT—pathologic correlation

Radiology

Resistance to airflow in patients with diffuse interstial lung disease

Am Rev Respir Dis