Antinuclear antibodies in primary pulmonary hypertension

S. Rich*, K. Kieras, K. Hart, B. M. Groves, J. D. Stobo, B. H. Brundage

*Corresponding author for this work

Research output: Contribution to journalEditorialpeer-review

137 Scopus citations


The association of positive antinuclear antibodies with the clinical and hemodynamic features of 43 patients with primary pulmonary hypertension and 16 patients with secondary pulmonary hypertension was investigated. Each patient had determinations of antinuclear antibodies using a KB cell substrate immunofluorescent test. Of the patients with primary pulmonary hypertension, 40% had positive antinuclear antibodies at titers of 1:80 dilutions or greater. There were no differences between patients with primary pulmonary hypertension and positive antinuclear antibodies compared with those with negative antinuclear antibodies in relation to clinical or hemodynamic status. A 6% incidence raie of antinuclear antibodies was found in patients with secondary pulmonary hypertension, similar to that in the normal population. The clinical, hemodynamic, serologic and histologic similarity between patients with primary pulmonary hypertension and those with unexplained pulmonary hypertension associated with collagen vascular disorders suggests that primary pulmonary hypertension in some patients may represent a collagen vascular disease confined to the lungs. The frequency of positive antinuclear antibody tests would place primary pulmonary hypertension between rheumatoid arthritis and scleroderma in the spectrum of collagen vascular diseases. Further studies are necessary, however, before one might expect that immunosuppressive therapy would be beneficial to these patients.

Original languageEnglish (US)
Pages (from-to)1307-1311
Number of pages5
JournalJournal of the American College of Cardiology
Issue number6
StatePublished - 1986

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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