A standardized histopathological classification for pulmonary vasculopathy in pulmonary arterial hypertension is proposed to document topographic variations and affected vascular segments.
A standardized histopathological classification of pulmonary vasculopathy in PAH may help improve understanding of the pathogenesis across various subsets of the disease.
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.
Pietra et al. (Tue,) conducted a review in Pulmonary arterial hypertension (PAH). A standardized histopathological classification for pulmonary vasculopathy in pulmonary arterial hypertension is proposed to document topographic variations and affected vascular segments.