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.
|Number of pages||5|
|Journal||Journal of Clinical Pathology|
|Publication status||Published - 1995|
- Idiopathic pulmonary fibrosis
ASJC Scopus subject areas
- Pathology and Forensic Medicine