PMC7152970_01
Chest computed tomography of the patient's native lungs demonstrating cystic changes, diffuse coarse reticular interstitial thickening with associated ground glass opacities.
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COPA syndrome included arthritis, biopsy-confirmed immune-mediated kidney disease, antineutrophil cytoplasmic antibody (ANCA) positivity, interstitial lung disease, diffuse alveolar hemorrhage, and progressive respiratory failure leading to mechanical ventilator support. Chest computed tomography demonstrated extensive cystic changes in the lungs, diffuse coarse reticular interstitial thickening, and ground glass opacities (Figure 1).
Histologic sections of the explanted (native) lungs show (a) fibrosing interstitial pneumonia with airspace enlargement (hematoxylin-eosin, original magnification 30x) and (b) alveolar hemorrhage with hemosiderin-laden macrophages and reactive type 2 cells (hematoxylin-eosin, original magnification 400x).
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Histologic sections of the explanted lungs showed a relatively uniform fibrosing interstitial pneumonia (nonusual interstitial pneumonia pattern) with honeycomb change and emphysema (Figure 2).