Pulmonary hypertension in a patient with mixed connective tissue disease: clinical and pathologic findings, and review of literature.

Abstract

A patient with pulmonary hypertension (PH) complicating mixed connective tissue disease (MCTD) is described. The patient died suddenly after catheterization documenting the pulmonary hypertension. Pathologic examination demonstrated marked medial hypertrophy and intimal proliferation of the pulmonary arterial vessels. Review of the literature revealed few and differing reports concerning the pathology of PH as seen in MCTD. Sudden death in patients with pulmonary hypertension undergoing invasive procedures is not an uncommon occurrence.