Lung
Other nonneoplastic disease
Plexiform arteriopathy
Author: Elliot Weisenberg, M.D.
Last author update: 1 December 2011
Last staff update: 30 September 2020
Copyright: 2003-2024, PathologyOutlines.com, Inc.
PubMed search: plexiform arteriopathy
Table of Contents
Definition / general | Microscopic (histologic) description | Microscopic (histologic) images | Differential diagnosisCite this page: Weisenberg E. Plexiform arteriopathy. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lungnontumorpulmonaryhyperplexart.html. Accessed December 3rd, 2024.
Definition / general
- Historical term based on lesions induced by congenital heart disease
- Heath and Edwards Classification from 1958 (modified below) still in widespread use
- Open lung biopsy in patients with congenital heart disease may be performed to determine if corrective vascular surgery will be beneficial
Microscopic (histologic) description
Grade I (early):
Grade II:
Grade III:
Grade IV to V:
Grade VI:
- Muscularization and media hypertrophy (> 7% of lumen) of pulmonary arteries
Grade II:
- Intimal hyperplasia causing attenuation of vascular lumen
Grade III:
- Subintimal fibrosis with onion ring appearance
- Marked reduplication of internal elastic membrane
- Arteries and arterioles resemble pipes
Grade IV to V:
- Dilation and plexiform lesions, aneurysmal dilation of small pulmonary arteries, plexiform and glomeruloid nodules
- Fibrin thrombi within plexiform lesion, old / new hemorrhage present
Grade VI:
- Uncommon, acute necrotizing arteritis with fibrinoid necrosis and acute inflammation of vessel wall, similar to polyarteritis nodosa
- Associated with extreme pulmonary hypertension
Microscopic (histologic) images
Images hosted on other servers:
Plexiform lesion
Differential diagnosis
- Secondary pulmonary hypertension: similar histology, different history
