PMC4401236_01
Representative chest tomographic (CT) images from the upper lobe (A), mid-lung region (B), and lower lobes (C and D) demonstrate bilateral, predominantly reticular, and peripheral opacities with associated traction bronchiectasis. The infiltrates are most prominent in the lung periphery and lower lung fields. Focal areas of early honeycombing may be seen in panel B (arrowhead).. Note: The sites marked by the asterisk (*) identify regions of the lung sampled at the time of surgical lung biopsy.
ccrpm-9-2015-023f1.jpg
PMC4401236_01_ccrpm-9-2015-023f1.jpg
f1-ccrpm-9-2015-023
Computed tomography (CT) of the chest showed subpleural reticular opacities most prominent at the bases with minimal areas of early honeycombing (Fig. 1). Given the unclear clinical picture, a surgical lung biopsy was performed: in consideration of the chest imaging findings (Fig. 1), wedge biopsies were obtained from the anterior aspect of the right upper lobe and the inferior aspects of the right middle and lower lobes.
Graphical depiction of change in vital capacity (VC) and DLCO from the time of diagnosis to the final follow-up. The y-axis refers to the percent predicted pulmonary function variable (VC or DLCO) relative to age, gender, and height-matched peers. The absolute VC and DLCO values at diagnosis (2.61 L and 13.4) and as of last follow-up (3.72 L and 17.9) are shown in the graph.
ccrpm-9-2015-023f2.jpg
PMC4401236_01_ccrpm-9-2015-023f2.jpg
f2-ccrpm-9-2015-023
Pulmonary function testing showed proportional reduction in forced vital capacity (FVC), forced expiratory volume in one second (FEV1; 69% and 73% predicted, respectively), and diffusing capacity for carbon monoxide (DLCO; 52% predicted) (Fig. 2). Her FEV1 also improved by 860 mL (31%), and her DLCO by 4.5 (>20%) (Fig. 2).
Surgical lung biopsy findings: (A) Low power view of the wedge biopsy from the right middle lobe lung shows mature fibrosis with honeycomb changes in the vicinity to alveolated lung parenchyma with preserved architecture and without fibrosis (regional heterogeneity). Within the mature fibrosis, there are fibroblastic foci (insert) (temporal heterogeneity) and areas of lymphoid infiltrate (B). These morphologic features are diagnostic of UIP. Magnifications x20 (A), x40 (B), and x200 (A, insert).
ccrpm-9-2015-023f3.jpg
PMC4401236_01_ccrpm-9-2015-023f3.jpg
f3-ccrpm-9-2015-023
Surgical lung biopsy showed predominantly mature fibrosis, honeycomb changes with regional heterogeneity, and fibroblast foci consistent with UIP: in addition, there were prominent inflammatory lymphoid infiltrates, mostly involving areas of fibrosis (Fig. 3).
Chest radiograph, performed at the time of last follow-up, demonstrating preserved lung volumes and peripheral reticular densities in the mid-lungs and lower lung fields.
ccrpm-9-2015-023f4.jpg
PMC4401236_01_ccrpm-9-2015-023f4.jpg
f4-ccrpm-9-2015-023
Chest X-ray performed at the most recent follow-up demonstrated normal lung volumes and mild interstitial prominence in the bases (Fig. 4).