scholarly journals Interferon and alternative activation of monocyte/macrophages in systemic sclerosis-associated pulmonary arterial hypertension

2011 ◽  
Vol 63 (6) ◽  
pp. 1718-1728 ◽  
Author(s):  
Romy B. Christmann ◽  
Everett Hayes ◽  
Sarah Pendergrass ◽  
Cristina Padilla ◽  
Giuseppina Farina ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Alternative Activation ◽  
Pulmonary Arterial

scholarly journals A Novel Multi-network Approach Reveals Tissue-specific Cellular Modulators of Fibrosis in Systemic Sclerosis, Pulmonary Fibrosis and Pulmonary Arterial Hypertension

2016 ◽  
Author(s):  
Jaclyn N Taroni ◽  
Casey S Greene ◽  
Viktor Martyanov ◽  
Tammara A Wood ◽  
Romy Christmann ◽  
...  
Keyword(s):  
Gene Expression ◽  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Pulmonary Fibrosis ◽  
Arterial Hypertension ◽  
Gene Expression Signature ◽  
Alternative Activation ◽  
Tissue Specific ◽  
Expression Signature ◽  
Pulmonary Arterial

We have used integrative genomics to determine if a common molecular mechanism underlies different clinical manifestations in systemic sclerosis (SSc), and the related conditions pulmonary fibrosis (PF) and pulmonary arterial hypertension (PAH). We identified a common pathogenic gene expression signature - an immune-fibrotic axis-indicative of pro-fibrotic macrophages in multiple affected tissues (skin, lung, esophagus and PBMCs) of SSc, PF, and PAH. We used this disease-associated signature to query tissue-specific functional genomic networks. This allowed us to identify common and tissue-specific pathology of SSc and related conditions. We rigorously contrasted the lung- and skin-specific gene-gene interaction networks to identify a distinct lung resident macrophage signature associated with lipid stimulation and alternative activation. In keeping with our network results, we find distinct macrophages alternative activation transcriptional programs in SSc-PF lung and in the skin of patients with an "inflammatory" SSc gene expression signature. Our results suggest that the innate immune system is central to SSc disease processes, but that subtle distinctions exist between tissues. Our approach provides a framework for examining molecular signatures of disease in fibrosis and autoimmune diseases and for leveraging publicly available data to understand common and tissue-specific disease processes in complex human diseases.

FRI0249 Pulmonary arterial hypertension in very early systemic sclerosis:

2013 ◽  
Vol 71 (Suppl 3) ◽  
pp. 398.2-398
Author(s):  
J. Sanchez ◽  
S. Jordan ◽  
J. Distler ◽  
B. Maurer ◽  
D. Huscher ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pulmonary Arterial ◽  
Early Systemic Sclerosis

Exercise-Induced Pulmonary Arterial Hypertension in Patients With Systemic Sclerosis*

CHEST Journal ◽  
2008 ◽  
Vol 134 (1) ◽  
pp. 146-151 ◽  
Author(s):  
Virginia Steen ◽  
Maria Chou ◽  
Victoria Shanmugam ◽  
Martin Mathias ◽  
Tunay Kuru ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pulmonary Arterial ◽  
Exercise Induced
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