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Updated: Jun 25, 2026
04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Resistin-like molecule-beta in scleroderma-associated pulmonary hypertension
Daniel J Angelini1, Qingning Su, Kazuyo Yamaji-Kegan
1Department of Anesthesiology and Critical Care Medicine, Johns Hopkins University School of Medicine, 720 Rutland Avenue, Ross 361, Baltimore, MD 21205, USA.
Summary
Resistin-like molecule-beta (RELM-beta) is elevated in scleroderma-associated pulmonary hypertension. This molecule may drive vascular remodeling and disease development in the lungs.
Area of Science:
- Pulmonary Medicine
- Vascular Biology
- Connective Tissue Diseases
Background:
- Scleroderma can lead to pulmonary hypertension and lung vascular remodeling.
- Molecular mechanisms driving scleroderma-associated pulmonary hypertension are poorly understood.
- Hypoxia-induced mitogenic factor (HIMF) is upregulated in a mouse model of pulmonary hypertension.
Purpose of the Study:
- To investigate the role of resistin-like molecule-beta (RELM-beta), the human homolog of HIMF, in scleroderma-associated pulmonary hypertension.
- To determine if RELM-beta expression is altered in lung tissue from patients with this condition.
Main Methods:
- Quantitative analysis of RELM-beta expression in lung tissue from scleroderma patients with pulmonary hypertension and healthy controls.
- Immunofluorescence colocalization to identify RELM-beta expressing cells in lung tissue.
- In vitro studies using recombinant RELM-beta on human pulmonary cells.
Main Results:
- RELM-beta is significantly upregulated in the lungs of patients with scleroderma-associated pulmonary hypertension compared to controls.
- RELM-beta is expressed in vascular endothelial cells, smooth muscle cells, macrophages, T cells, and myofibroblast-like cells within remodeled lung vasculature and plexiform lesions.
- Recombinant RELM-beta promotes proliferation and ERK1/2 activation in human pulmonary endothelial and smooth muscle cells.
Conclusions:
- RELM-beta is implicated in the pathogenesis of scleroderma-associated pulmonary hypertension.
- RELM-beta may contribute to vascular remodeling and disease progression through its effects on pulmonary cells.
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