Presentation
Chronic progressive shortness of breath.
Patient Data
Severe extent fibrotic lung disease, with the peripheral, basal predominant coarse reticulation, traction bronchiectasis and honeycombing. Small component of perilobular/subpleural confluent fibrosis at the apices. Moderate progression in both degree of coarseness and extent of lung involvement since prior scan from an year ago (not shown).
Enlarged main pulmonary artery (37 mm). Mild right heart dilatation. No pericardial or pleural effusion. Small reactive mediastinal lymph nodes as before.
Unchanged mildly dilated esophagus. No hiatus hernia. Previous cholecystectomy noted. No destructive bone lesion.
Specimen Description: Native left lung
Macroscopic Description: Labeled "Native left lung". A left lung, 205 x 160 x 65 mm, comprising upper lobe, 175 x 100 x 75 mm, and lower lobe, 180 x 95 x 85 mm. The pleural surface is tan, with minimal anthracotic pigment, and a nodular, cobblestoned appearance. The cut section shows firm white fibrous tissue in a subpleural distribution, predominantly affecting the base of the lower lobe, and inferior portions of the upper lobe. There is sparing of the central portions of the lung, and apex. There is cystic honeycombing of the affected fibrous areas. Bronchioles are dilated, and extend to the peripheries. There is a thin fibrous cap at the apex, 32 x 32 x 5 mm. There are no endobronchial lesions. There is mild pale yellow atherosclerotic plaque within pulmonary arterial branches. No pulmonary emboli are seen.
Microscopic Description: Sections of lung show areas of relatively normal-looking alveolar parenchyma alternating with areas of dense fibrosis within which there is honeycombing. Fibroblastic foci are identified. Fibrosis is more prominent within subpleural areas and is also seen to a lesser degree in some peribronchial areas. There is arterial atherosclerosis. A few benign lymph nodes are identified. There is no evidence of malignancy.
Diagnostic Opinion: Native left lung with changes of usual interstitial pneumonia.
Case Discussion
This case shows typical UIP pattern on imaging:
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distribution
basal predominant
subpleural predominant
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features
honeycombing
reticulation with peripheral bronchiectasis or bronchiectasis
absence of features suggesting an alternative diagnosisÂ
The presence of this pattern, in the correct clinical setting, permits a confident diagnosis of IPF (idiopathic pulmonary fibrosis), which was later confirmed on pathology.
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