CTICasesIn Situ Pulmonary Artery Thrombosis in Dermatomyositis with Pulmonary Fibrosis
Imaging findings
Chest CT demonstrates severe pulmonary fibrosis, emphysema, and pulmonary hypertension characterized by right ventricular hypertrophy and septal flattening. Non-obstructive, wall-layering, eccentric in situ thrombus is visible in the right middle and lower lobe pulmonary arteries, without distal occlusions or perfusion defects on dual-energy CT.
Key takeaways
In situ pulmonary artery thrombosis is a well-recognized complication of severe chronic fibrotic lung diseases (such as systemic sclerosis or dermatomyositis-related interstitial lung disease). It is non-obstructive, lacks the characteristic findings of thromboembolic CTEPH, and does not benefit from surgical thromboendarterectomy as the underlying parenchymal destruction is irreversible.
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