ICD-11 classes
12 Diseases of the respiratory system
Respiratory diseases principally affecting the lung...
CB03 — Idiopathic interstitial pneumonitis
CB03.1 — Combined pulmonary fibrosis and emphysema syndrome

ICD-11 CB03.1 — Combined pulmonary fibrosis and emphysema syndrome

Combined pulmonary fibrosis and emphysema (CPFE) is a syndrome of combined emphysema of the upper lobes and fibrosis of the lower lobes defined on chest computed tomography, and characterised by subnormal spirometry, severe impairment of gas exchange, high prevalence of pulmonary hypertension, and poor survival.Characteristic functional profile of CPEF is strongly impaired carbon monoxide diffusing capacity of the lung, and hypoxaemia at exercise, with preserved lung volumes. Despite subnormal spirometry, which may be responsiblefor its under recognition, CPFE is a severe entity. The presence of pulmonary arterial hypertension at diagnosis is a critical determinant of prognosis.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.