Staging Criteria of Powars et al for Sickle Chronic Lung Disease

Parameter

Stage 1

Stage 2

clinical findings

chronic cough; recurrent substernal chest pain

moderate chest pain

arterial blood gases

normal oxygen saturation

normal oxygen saturation

chest X-ray

changes suggestive of increased interstitial markings; hyperexpansion; decreased distal pulmonary vascularity

diffuse, fine interstitial fibrosis in all lobes

pulmonary function

mild decrease, at 80% of predicted

moderate decrease at 60% of predicted

ECG and Echocardiography

left ventricular preponderance

balanced ventricular hypertrophy

pulmonary artery pressure

normal

normal

Parameter

Stage 3

Stage 4

clinical findings

severe midline chest pain ("crushing")

severe and prolonged chest pain

arterial blood gases

mild to moderate hypoxia (PaO2 70 mm Hg while stable)

severe hypoxia (PaO2 60 mm Hg while stable)

chest X-ray

pulmonary fibrosis

severe pulmonary fibrosis

pulmonary function

severe decrease, at 40% of predicted

marked decrease; patient unable to perform testing; dyspnea at rest

ECG and Echocardiography

right ventricular hypertrophy and right atrial dilation

severe right ventricular and right atrial hypertrophy' cor pulmonale

pulmonary artery pressure

borderline elevation or high normal

pulmonary hypertension