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Plexiform lesions in pulmonary arterial hypertension composition, architecture, and microenvironment
Danny Jonigk1, Heiko Golpon, Clemens L Bockmeyer
1Institute of Pathology, Hannover Medical School, Hanover, Germany. jonigk.danny@mh-hannover.de
The American Journal of Pathology
|June 28, 2011
Summary
Plexiform lesions in pulmonary arterial hypertension (PAH) have a unique cellular makeup and microenvironment, distinct from neoplastic angiogenesis. These findings clarify the pathology of PAH vascular remodeling.
Area of Science:
- Vascular Biology
- Pulmonary Hypertension Research
- Cellular Pathology
Background:
- Pulmonary arterial hypertension (PAH) is a severe condition with high mortality.
- Plexiform lesions (PLs) are characteristic vascular abnormalities in PAH, with debated origins and functions.
- PLs share some features with glomeruloid-like lesions (GLLs) found in glioblastomas, prompting comparison.
Purpose of the Study:
- To investigate the cellular composition and molecular characteristics of plexiform lesions in PAH.
- To compare PLs in PAH with glomeruloid-like lesions (GLLs) in glioblastomas.
- To determine the relevance of neoplastic angiogenesis models for studying PAH vasculopathy.
Main Methods:
- In situ techniques including fluorescent immunohistochemistry staining.
- Three-dimensional reconstruction and laser microdissection.
- Compartment-specific mRNA expression analysis in lung tissue from PAH patients and glioblastoma samples.
Main Results:
- PLs feature continuously proliferating endothelium and a myogenic interstitium.
- Upregulation of remodeling genes (HIF1α, TGF-β1, VEGF) and sprouting markers (cKIT, NOTCH) observed in PLs.
- Significant differences in cellular composition and signaling between PLs and GLLs were identified.
Conclusions:
- Plexiform lesions in PAH possess a distinct cellular composition and microenvironment.
- These unique features define the plexiform phenotype and differentiate them from other vascular remodeling processes in PAH.
- Neoplastic angiogenesis models offer limited utility for studying plexiform vasculopathy.
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