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CFPE is a distinct but under-recognized and common syndrome with a characteristic presentation.Further studies are needed to ascertain the etiology, morbidity, and mortality of CPEF with or without PH, and to evaluate novel management options.
Patients could not have diabetes, cardiovascular disease, renal disease or other serious conditions.
Enrolled patients attended two screening visits, and those patients with SBP between 160 and 199 mm Hg were stratified into a separate subgroup to make sure even numbers were distributed in the three study groups.
The association of emphysema with IPF has been termed the combined pulmonary fibrosis and emphysema (CPFE) syndrome [4, 5] to account for the characteristic clinical, functional, imaging, and outcome features.
Characteristics of patients with CPFE syndrome include male predominance, tobacco smoking, severe dyspnoea, subnormal spirometry findings, severely impaired transfer capacity for carbon monoxide [4, 6, 7], hypoxemia upon exercise, high frequency of paraseptal emphysema, and a high probability (30–50%) of severe pulmonary hypertension (PH) impacting upon an already poor prognosis [2, 4].
There is an increasing awareness of comorbid conditions frequently associated with idiopathic pulmonary fibrosis (IPF), including emphysema, cardiovascular disease, thromboembolic disease, and obstructive sleep apnoea.
Recent retrospective data suggest that 21 to 33% of patients with IPF may have co-existing emphysema [1–3].
JJ is a 70 year old patient with hypertension (HTN) and hyperlipidemia.
He has difficulty remembering to take his blood pressure (BP) medication because his BP doesn't really bother him. This HMO has a number of different care management, case management, and disease management programs, particularly for their older members.