CTICasesPlexiform Arteriopathy in Scleroderma-Associated PAH
[...]. Plexiform Arteriopathy in Scleroderma-Associated PAH. CTICases. Published July 18, 2020. https://str-case-explorer.pages.dev/c/cxtjn
Imaging findings
CT in a 64-year-old female with scleroderma and pulmonary hypertension shows a dilated main pulmonary artery and diffuse, smudgy centrilobular ground-glass nodules representing plexiform lesions (arteriopathy) rather than PVOD/PCH, with slightly elevated wedge pressures.
Key takeaways
Centrilobular ground-glass nodules in patients with connective tissue disease-associated pulmonary hypertension often correspond to plexiform lesions of plexiform arteriopathy. They must be distinguished from PVOD, which typically shows interlobular septal lines and pleural effusions.
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