A possible contribution of lipocalin-2 to the development of dermal fibrosis, pulmonary vascular involvement and renal dysfunction in systemic sclerosis

• DOI: 10.1111/bjd.13779
• Publication Date: 2015-03-17T14:21:57Z
• Authors: T. Takahashi; Y. Asano; S. Noda; N. Aozasa; K. Akamata; T. Taniguchi; Y. Ichimura; T. Toyama; H. Sumida; Y. Kuwano; Y. Tada; M. Sugaya; T. Kadono; S. Sato
• Keywords: Scleroderma (fungus)

Background Lipocalin-2 is an adipocytokine implicated in apoptosis, innate immunity, angiogenesis, and the development of chronic kidney disease. Objectives To investigate role lipocalin-2 systemic sclerosis (SSc). Materials methods Serum levels were determined by enzyme-linked immunosorbent assay 50 patients with SSc 19 healthy subjects. expression was evaluated skin bleomycin (BLM)-treated mice Fli1-deficient endothelial cells reverse transcriptase-real time polymerase chain reaction, immunoblotting and/or immunohistochemistry. Results Although serum comparable between controls, prevalence scleroderma renal crisis significantly higher elevated than those normal levels. Furthermore, inversely correlated estimated glomerular filtration rate dysfunction. Among function, positively score diffuse cutaneous disease duration < 3 years right ventricular systolic pressure total SSc. Importantly, lesional skin, increased dermal fibroblasts cells. In BLM-treated mice, highly expressed fibroblasts, but not On other hand, deficiency transcription factor Fli1, which vasculopathy, induced cultivated Conclusions may be involved dysfunction fibrosis Dysregulated matrix metalloproteinase-9/lipocalin-2-dependent angiogenesis due to Fli1 contribute pulmonary arterial hypertension associated