Endothelin-1 in idiopathic pulmonary fibrosis

M. Uguccioni, L. Pulsatelli, B. Grigolo, A. Facchini, C. Cinti, M. Fabbri, G. Gasbarrini, R. Meliconi

Research output: Contribution to journalArticlepeer-review


Aims: To evaluate whether endothelin-1 is involved in the pathology of idiopathic pulmonary fibrosis (IPF). Methods: Plasma endothelin-1 concentrations were evaluated in 37 patients with IPF and 27 normal controls by radioimmunoassay. In addition, expression of endothelin-1 in lung tissue was evaluated in biopsy specimens obtained from four patients with IPF. Three biopsy specimens of normal lung were used as controls. Endothelin-1 immunoreactivity was detected using immunohistochemistry. Results: Elevated endothelin-1 plasma concentrations were found in patients with IPF compared with controls and a positive correlation was found with duration of disease. No significant difference was observed between treated and untreated patients with IPF. Increased endothelin-1 immunoreactivity was found in lungs of three of four patients with IPF. Endothelin-1 positive cells consisted mainly of small vessel endothelial cells. Some scattered macrophages were also positive. Conclusions: Elevated plasma concentrations and expression of endothelin-1 in lung tissue are suggestive of increased production of endothelin-1 in at least a proportion of patients with IPF. Consequently, endothelin-1 activity could play a role in the fibrogenic process of the disease.

Original languageEnglish
Pages (from-to)330-334
Number of pages5
JournalJournal of Clinical Pathology
Issue number4
Publication statusPublished - 1995


  • Endothelin
  • Idiopathic pulmonary fibrosis

ASJC Scopus subject areas

  • Pathology and Forensic Medicine


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