Which histologic findings are characteristic of pulmonary arterial hypertension (PAH)?

Updated: Apr 25, 2018
  • Author: Kristin E Schwab, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Answer

The histopathologic lesions in patients with pulmonary arterial hypertension (PAH) are the result of long-standing pulmonary hypertension rather than a consequence of different causes.

The plexiform lesion is observed in patients with all types of PAH. These lesions consist of medial hypertrophy, eccentric or concentric laminar intimal proliferation and fibrosis, fibrinoid degeneration, and thrombotic lesions. Fresh or organized and recanalized thrombi may also be present. Diverse types of intimal and muscular lesions of the small muscular arteries may cause the clinical syndrome of PAH, and a plexiform lesion reflecting the abrupt onset of PAH is likely, rather than the lesion being a distinctive cause.


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