Pathogenesis and evolution of plexiform lesions in pulmonary hypertension associated with scleroderma and human immunodeficiency virus infection

Hum Pathol. 1997 Apr;28(4):434-42. doi: 10.1016/s0046-8177(97)90032-0.


Patients with primary pulmonary hypertension develop vascular lesions characterized by proliferated blood channels, the so-called plexiform lesions. These lesions are often associated with concentric intimal obliteration of pulmonary vessels. We report that the lungs of three patients with scleroderma-associated pulmonary hypertension showed a predominance of obliterative-concentric lesions, with relatively few plexiform or combined lesions. In contrast, plexiform lesions predominated in the lungs obtained from three patients with human immunodeficiency virus (HIV)-associated pulmonary hypertension; pure obliterative-concentric lesions were infrequent. Both plexiform and concentric obliterative lesions stained strongly positive for the endothelial cell marker factor VIII-related antigen. Muscle-specific actin immunostaining highlighted the smooth muscle cells of the tunica media of plexiform vessels, but not the luminal layers of the concentric-obliterative lesions. Proliferating cells, as determined by immunostaining with the MIB-1 antibody, were only detected in the plexiform vascular lesions. We postulate that concentric-obliterative lesions and plexiform lesions are temporally and etiologically related. A scaffolding of proliferating endothelial cells could be the common denominator of both lesions. Our hypothesis that there exists a chronological continuum, proceeding from early, proliferative plexiform lesions to late, nonproliferative concentric-obliterative lesions in primary and secondary pulmonary hypertension, may lead to better targeted treatment strategies and disease classification.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Actins / analysis
  • Adult
  • Antigens, CD / analysis
  • Antigens, Differentiation, Myelomonocytic / analysis
  • CD3 Complex / analysis
  • Cell Division
  • Endothelium, Vascular / chemistry
  • Endothelium, Vascular / immunology
  • Endothelium, Vascular / pathology
  • Factor VIII / analysis
  • Female
  • HIV Infections / complications*
  • HIV Infections / immunology
  • Humans
  • Hypertension, Pulmonary / etiology*
  • Hypertension, Pulmonary / immunology
  • Hypertension, Pulmonary / pathology*
  • Immunohistochemistry
  • Ki-67 Antigen / analysis
  • Leukocyte Common Antigens / analysis
  • Lung / blood supply
  • Lung / chemistry
  • Lung / immunology
  • Lung / pathology*
  • Male
  • Middle Aged
  • Muscle, Smooth, Vascular / chemistry
  • Muscle, Smooth, Vascular / pathology
  • Scleroderma, Systemic / complications*
  • Scleroderma, Systemic / immunology


  • Actins
  • Antigens, CD
  • Antigens, Differentiation, Myelomonocytic
  • CD3 Complex
  • CD68 antigen, human
  • Ki-67 Antigen
  • Factor VIII
  • Leukocyte Common Antigens