Key result
Novel PAH histopathological classification standardizes reporting across affected vascular segments.
Why the study?
Histopathological vascular changes in PAH are qualitatively similar but vary quantitatively in distribution and prevalence, necessitating standardized documentation to understand pathogenesis.
A proposed histopathological classification aims to standardize the documentation of pulmonary vasculopathy in pulmonary arterial hypertension to better understand its pathogenesis.
Promotes uniform histopathological evaluation of PAH; leaves open integration into risk models or therapeutic trials.
Pulmonary arterial hypertension (PAH) includes various forms of pulmonary hypertension of different etiology but similar clinical presentation and functional derangement. Histopathological vascular changes in all forms of PAH are qualitatively similar but with quantitative differences in the distribution and prevalence of pathological changes in various portions of the pulmonary vascular bed. The documentation of these topographic variations in the response of the pulmonary vasculature to injury may be important to understand the pathogenesis of the various subsets of PAH. To standardize the precise histopathological documentation of the pulmonary vasculopathy in PAH we propose a histopathological classification that includes both the predominant segment of the pulmonary vasculature affected and the possible coexistence of pathological changes in other vascular segments.
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Pietra et al. (2004) conducted a review in Pulmonary arterial hypertension. A proposed histopathological classification for pulmonary arterial hypertension aims to standardize the documentation of pulmonary vasculopathy by detailing affected vascular segments.
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