Chronic pulmonary disorders in sickle cell disease: findings at thin-section CT.

Abstract

To characterize the non-acute abnormalities seen at computed tomography (CT) in patients with sickle cell (SC) disease and a prior history of acute chest syndrome (ACS)-pneumonia. Twenty-nine patients with SC disease who had experienced one to more than 10 (median, six) previous episodes of ACS-pneumonia were prospectively studied with thin-section CT of the thorax. Scans were graded for interstitial disease and assigned a disease index ranging from 0 to 3. Twenty-four patients underwent pulmonary function tests (PFTs) and measurement of their blood gasses. Twelve of the 29 patients (41%) had significant interstitial disease that was multifocal. A correlation was found between the disease index and number of episodes of ACS-pneumonia (P = .02) but not between the disease index and PFT results. Thin-section CT demonstrates significant multifocal interstitial lung abnormalities in 41% of selected patients with SC disease. The pattern is most consistent with scarring from episodes of infarction or infection.