PMC2779311_01
Computed tomography (CT) scans of the patient (A) and the patient's sister (B), showing diffuse fibrosis and subpleural consolidations in both lungs.
jkms-20-1066-g001.jpg
PMC2779311_01_jkms-20-1066-g001.jpg
F1
Chest CT (Fig. 1A) demonstrated diffuse fibrosis on the medial portion of both lung fields and subpleural consolidation along both lateral pleura. Based on the similar findings from chest CT (Fig. 1B), the sister of the patient had been diagnosed with uncharacterized ILD, and died 5 months after diagnosis.
Interstitial pneumonia with lymphoplasmacytic infiltration and variable amount of collagen fibrosis resulted in the thickening of the interstitium. Most alveoli also show variable amount of intra-alveolar macrophage accumulation and hyperplasia of the alveolar pneumocytes. (A) Hematoxylin-eosin stain, x100, (B) Masson's trichrome stain, x100.
jkms-20-1066-g002.jpg
PMC2779311_01_jkms-20-1066-g002.jpg
F2
Surgical open lung biopsy (Fig. 2) was performed at 22 months of age.