PMC10115523_01
CT chest pretransplant (28/04/2015) demonstrating a UIP pattern of IPF.
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Investigations included a negative autoimmune screen and computed tomography (CT) imaging which demonstrated interstitial thickening, honeycombing, and traction bronchiectasis (Figure 1) suggestive of a radiological UIP pattern.
(a) Histopathology specimens demonstrating extensive areas of established fibrosis and extensive remodelling producing honeycombing. (b) Histopathology section demonstrating subpleural and paraseptal predominance of the fibrosis with small areas of relative preservation without fibrosis. (c) Histopathology section demonstrating the presence of fibroblast foci.
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Histopathology specimens demonstrated extensive areas of established fibrosis and extensive remodelling producing end-stage lung disease with honeycombing (Figure 2), especially in a subpleural and septal distribution, with small areas of relative preservation without fibrosis (Figure 2(b)) and scattered fibroblastic foci (Figure 2(c)), all consistent with UIP.
PMC10115523_02
CT chest imaging 2 years (12/09/2018) posttransplant.
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Graph of our patient's FEV1 trend from pretransplant in June 2015 to posttransplant up to July 2020.
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(a) Histopathology showing diffuse, uniform macrophage accumulation within alveolar spaces. (b) Histopathology highlighted on immunochemistry for CD68. (c) Histopathology demonstrating focal interstitial thickening and inflammatory changes.
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The sections of the peripheral lung from both lobes showed a diffuse alveolar filling defect with the expansion of the alveolar spaces by numerous alveolar macrophages, a proportion of which contained haemosiderin pigment (Figure 5(a)). These cells were highlighted on an immunoperoxidase stain for CD68 (Figure 5(b)). This was accompanied by focal regions of interstitial fibrosis with the thickening and early distortion of the alveolar septae (Figure 5(c)), with multifocal regions of type 2 pneumocyte hyperplasia and small foci of megakaryocytes, as well as mild interstitial infiltrate within the alveolar septae that included scattered eosinophils but no significant lymphocytic aggregates or prominent germinal centres all consistent with DIP.