PMID- 20973920 OWN - NLM STAT- MEDLINE DCOM- 20110616 LR - 20211020 IS - 1752-8062 (Electronic) IS - 1752-8054 (Print) IS - 1752-8054 (Linking) VI - 3 IP - 5 DP - 2010 Oct TI - Altered immune phenotype in peripheral blood cells of patients with scleroderma-associated pulmonary hypertension. PG - 210-8 LID - 10.1111/j.1752-8062.2010.00218.x [doi] AB - Pulmonary arterial hypertension is a common and fatal complication of scleroderma that may involve inflammatory and autoimmune mechanisms. Alterations in the gene expression of peripheral blood mononuclear cells have been previously described in patients with pulmonary arterial hypertension. Our goal is to identify differentially expressed genes in peripheral blood mononuclear cells in scleroderma patients with and without pulmonary hypertension as biomarkers of disease. Gene expression analysis was performed on a Microarray Cohort of scleroderma patients with (n = 10) and without (n = 10) pulmonary hypertension. Differentially expressed genes were confirmed in the Microarray Cohort and validated in a Validation Cohort of scleroderma patients with (n = 15) and without (n = 19) pulmonary hypertension by RT-qPCR. We identified inflammatory and immune-related genes including interleukin-7 receptor (IL-7R) and chemokine receptor 7 as differentially expressed in patients with scleroderma-associated pulmonary hypertension. Flow cytometry confirmed decreased expression of IL-7R on circulating CD4+ T-cells from scleroderma patients with pulmonary hypertension. Differences exist in the expression of inflammatory and immune-related genes in peripheral blood cells from patients with scleroderma-related pulmonary hypertension compared to those with normal pulmonary artery pressures. These findings may have implications as biomarkers to screen at-risk populations for early diagnosis and provide insight into mechanisms of scleroderma-related pulmonary hypertension. CI - (c) 2010 Wiley Periodicals, Inc. FAU - Risbano, Michael G AU - Risbano MG AD - Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Denver, Aurora, USA. onabsir@gmail.com FAU - Meadows, Christina A AU - Meadows CA FAU - Coldren, Christopher D AU - Coldren CD FAU - Jenkins, Tiffany J AU - Jenkins TJ FAU - Edwards, Michael G AU - Edwards MG FAU - Collier, David AU - Collier D FAU - Huber, Wendy AU - Huber W FAU - Mack, Douglas G AU - Mack DG FAU - Fontenot, Andrew P AU - Fontenot AP FAU - Geraci, Mark W AU - Geraci MW FAU - Bull, Todd M AU - Bull TM LA - eng GR - K08 HL072858/HL/NHLBI NIH HHS/United States GR - L30 HL097867/HL/NHLBI NIH HHS/United States GR - K08 HLO72858-01A2/PHS HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PL - United States TA - Clin Transl Sci JT - Clinical and translational science JID - 101474067 RN - 0 (Receptors, Interleukin-7) SB - IM EIN - Clin Transl Sci. 2010 Dec;3(6):340. Hunt, James [added] CIN - Clin Transl Sci. 2011 Feb;4(1):72. PMID: 21348960 MH - Blood Cells/*immunology MH - CD4-Positive T-Lymphocytes/immunology MH - Cluster Analysis MH - Cohort Studies MH - Demography MH - Familial Primary Pulmonary Hypertension MH - Female MH - Flow Cytometry MH - Hemodynamics MH - Humans MH - Hypertension, Pulmonary/blood/etiology/genetics/immunology MH - Male MH - Middle Aged MH - Oligonucleotide Array Sequence Analysis MH - Phenotype MH - Receptors, Interleukin-7/immunology MH - Reproducibility of Results MH - Reverse Transcriptase Polymerase Chain Reaction MH - Scleroderma, Systemic/*blood/*complications/genetics/immunology PMC - PMC2966033 MID - NIHMS224993 EDAT- 2010/10/27 06:00 MHDA- 2011/06/17 06:00 PMCR- 2010/10/01 CRDT- 2010/10/27 06:00 PHST- 2010/10/27 06:00 [entrez] PHST- 2010/10/27 06:00 [pubmed] PHST- 2011/06/17 06:00 [medline] PHST- 2010/10/01 00:00 [pmc-release] AID - CTS218 [pii] AID - 10.1111/j.1752-8062.2010.00218.x [doi] PST - ppublish SO - Clin Transl Sci. 2010 Oct;3(5):210-8. doi: 10.1111/j.1752-8062.2010.00218.x.