Abstract
Recent studies have described the combination of both pulmonary emphysema and idiopathic interstitial lung disease (ILDs) by means of high-resolution computed axial tomography (HRCT). Definition of this syndrome was first named by Cottin as combined pulmonary fibrosis and emphysema (CPFE). Functional and radiological findings have showed that these patients are suffering from severe breathlessness, but whose pulmonary functional tests revealed no signs of obstruction, normal static lung volumes, and depressed DLco, most with a history of smoking [1] [2]. The radiological and endoscopic studies especially show that these patients have both areas of upper-lobe predominant emphysema and lesions compatible with fibrosis in both lung bases [3]. No prior research has reported any cases of such condition in person with no prior history of smoking as well as long-term high-dose of glucocorticoid therapy. In this case report, we discuss the presentation, diagnosis, and management of a 53-year-old non-smoker with increasing shortness of breath with a long-term high-dose of glucocorticoid therapy discovered to have an abnormal variant or presentation of CPFE. The cause of disease was attributed to a certain history of smoking in most studies; other potential risk factors have yet to be properly analyzed. This clinical report features a special case about the problem and solution surrounding this issue.
Highlights
The descriptions of combined pulmonary fibrosis and emphysema (CPFE) include some certain features as male sex, a history of cigarette smoking, relatively preserved spirometric values, and decreased diffusing capacity of lung for carbon monoxide (DLco) [6]
Diagnosis of CPFE on high-resolution computed axial tomography (HRCT) is still the certain method to assess the degree of emphysema and fibrosis, which may make the progress effective and accurate [9]
In this case presentation unlike typical CPFE, emphysema was found in the lower lobe on this patient, CT scan was unremarkable for emphysematous changes, lung volume changes were present, and the patient had a long-term high-dose of glucocorticoid therapy instead of smoking history
Summary
Studies have shown that patients with CPFE are mostly presented with tobacco exposure They are predominantly male, with a history of heavy tobacco exposure, and usually present with severe breathlessness and cough. Physical examination reveals “Velcro” crackles at the lung bases and digital clubbing [3]. In this case, the patients were discovered with non-smoking history, but a long-term high-dose of glucocorticoid therapy in treating gouty arthritis. This brings into a new question, whether there is an undiscovered risk factor or a new condition, which presents differently and may be the result of another risk factor, other than smoking
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