PURPOSE: Coincidental affliction with Idiopathic Pulmonary Fibrosis (IPF) and Emphysema was first described in 19901. The syndrome is characterized by radiological evidence of emphysema in the upper lobes and interstitial fibrosis and honeycombing involving the lower lobes, severe dyspnea and hypoxemia, severely reduced DLCO, and significant Pulmonary Hypertension (PH)2. Despite the parenchymal involvement, the symptoms are largely attributable to severe PH. We aim to describe a series of 3 patients with findings suggestive of CPFE and rapid decline after diagnosis of PH.
METHODS: Retrospective chart review of 3 patients with CPEF including their demographic information, pulmonary functions, radiological, and echocardiography reports.
RESULTS: We present three males, all with >50 pack year smoking history, with a median age of 71 years. They had a mean FEV1 of 1.66L, a mean TLC of 73 % and mean DLCO was 29%. CT scans demonstrated upper zone emphysema with basal fibrosis with mean pulmonary artery diameter of 4.12 cm. We had cardiac data on two patients as case 3 declined the studies. Echo showed both indirect evidence of PH and had a mean RVSP of 65 mmHg. The median survival after the diagnosis of PH was 6mths.
CONCLUSION: CPFE is a distinct entity from IPF and is characterized by radiological evidence of emphysema in the upper zones and interstitial fibrosis involving the lower zones. Near normal Lung volumes with severe diffusion abnormalities is common. Pulmonary hypertension is more severe in CPFE than either IPF or emphysema alone and its onset is a harbinger of poor prognosis and increased mortality. Is pulmonary hypertension simply an additive effect of two disease processes independently associated with pulmonary hypertension or is there a common process(s) in genetically predisposed individuals, possibly mediated through chronic inflammation induced by cigarette smoke, is unknown?.
CLINICAL IMPLICATIONS: CFPE is an underecognized but common syndrome in evolution. Further studies are needed to ascertain the etiology, morbidity, mortality and management of CPEF, with or without PH.
DISCLOSURE: Nikhil Meena, No Financial Disclosure Information; No Product/Research Disclosure Information