Mixed connective tissue disease with pulmonary hypertension: a clinical and pathological study

J Rheumatol. 1987 Aug;14(4):826-30.


Clinical and autopsy findings of 2 cases with mixed connective tissue disease (MCTD) associated with severe pulmonary hypertension are reported. Both cases showed marked intimal and medial thickening in small and medium sized pulmonary arteries. In one of the cases plexiform lesions, endarteritis obliterans, and fibrous intimal thickening of the pulmonary veins were recognized. In some cases with MCTD, pulmonary hypertension develops due to pulmonary vascular lesions which are similar to those of primary pulmonary hypertension.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Female
  • Humans
  • Hypertension, Pulmonary / etiology*
  • Hypertension, Pulmonary / pathology
  • Male
  • Middle Aged
  • Mixed Connective Tissue Disease / complications*
  • Mixed Connective Tissue Disease / pathology
  • Pulmonary Artery / pathology