Systemic sclerosis (SSc) is a rare, complex rheumatic disease involving multiple organ systems with a frequently fatal outcome. It remains one of the most difficult rheumatic disease to manage, with limited effective therapies. There are several impediments to more rapid advances in understanding SSc pathogenesis and finding new treatments. First, the relative rarity of SSc and need of these patients for specialty clinical care has concentrated translational studies in select institutions having an adequate patient base and a program for sample/tissue collection. Thus many investigators have little or no access to patient samples/tissues. Even for investigators seeing relatively large numbers of patients, large sample sizes for proving associations between clinical disease and pathogenic features under study can prove elusive. Second, the heterogeneity of patient presentation and disease progression leads to fragmented approaches to understanding pathogenesis, different investigators studying different disease manifestations, for example, skin fibrosis compared to pulmonary arterial hypertension. Third, advanced technologies, such as microarray and proteomics are applied using different platforms, rendering interpretation of studies across different sites difficult. Empowered by a Consortia of SSc investigators formed to address these problems, this Core Center will leverage existing institutional resources to form four cores, providing patient samples and common platforms for advanced technologies designed to serve the broad clinical, translational basic scientific interests of Consortia Investigators. The Administrative Core (Core A), will provide structure, communication and enrichment of Core Investigators. Two cores (Cores B and C) will array pathological specimens from skin and lung, respectively, from SSc patients for rapid immunohistochemical evaluation of target proteins. Two cores will provide Consortia Investigators access to powerful Proteomic (Core D) and Microarray (Core E) technologies. All Cores will additionally use economy-of-scale and Core funding to provide services at a fraction of normal cost. In addition, the common platform and clinical data collected across cores will provide a large, common database for understanding clinical-pathological associations.

Public Health Relevance

SSc is a devastating rheumatic condition often leading to death. Because the disease is uncommon, it has been difficult to organize resources and obtain adequate sample numbers to understand disease pathogenesis. This Core Center will provide a mechanism for a consortia of investigators with strong records of accomplishment in SSc research to utilize common cores and platforms for high technology analyses.

National Institute of Health (NIH)
National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
Center Core Grants (P30)
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Special Emphasis Panel (ZAR1-MLB (M1))
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Mancini, Marie
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Boston University
Internal Medicine/Medicine
Schools of Medicine
United States
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Urso, Katia; Alvarez, David; Cremasco, Viviana et al. (2016) IL4RA on lymphatic endothelial cells promotes T cell egress during sclerodermatous graft versus host disease. JCI Insight 1:
Ziemek, Jessica; Man, Ada; Hinchcliff, Monique et al. (2016) The relationship between skin symptoms and the scleroderma modification of the health assessment questionnaire, the modified Rodnan skin score, and skin pathology in patients with systemic sclerosis. Rheumatology (Oxford) 55:911-7
Nazari, Banafsheh; Rice, Lisa M; Stifano, Giuseppina et al. (2016) Altered Dermal Fibroblasts in Systemic Sclerosis Display Podoplanin and CD90. Am J Pathol 186:2650-64
Martyanov, Viktor; Whitfield, Michael L (2016) Molecular stratification and precision medicine in systemic sclerosis from genomic and proteomic data. Curr Opin Rheumatol 28:83-8
Mendoza, Fabian A; Piera-Velazquez, Sonsoles; Farber, John L et al. (2016) Endothelial Cells Expressing Endothelial and Mesenchymal Cell Gene Products in Lung Tissue From Patients With Systemic Sclerosis-Associated Interstitial Lung Disease. Arthritis Rheumatol 68:210-7
Ahluwalia, Neil; Grasberger, Paula E; Mugo, Brian M et al. (2016) Fibrogenic Lung Injury Induces Non-Cell-Autonomous Fibroblast Invasion. Am J Respir Cell Mol Biol 54:831-42
Christmann, Romy B; Wooten, Alicia; Sampaio-Barros, Percival et al. (2016) miR-155 in the progression of lung fibrosis in systemic sclerosis. Arthritis Res Ther 18:155
Ramos, Paula S; Silver, Richard M; Feghali-Bostwick, Carol A (2015) Genetics of systemic sclerosis: recent advances. Curr Opin Rheumatol 27:521-9
Rice, Lisa M; Padilla, Cristina M; McLaughlin, Sarah R et al. (2015) Fresolimumab treatment decreases biomarkers and improves clinical symptoms in systemic sclerosis patients. J Clin Invest 125:2795-807
Roach, Katy M; Feghali-Bostwick, Carol A; Amrani, Yassine et al. (2015) Lipoxin A4 Attenuates Constitutive and TGF-β1-Dependent Profibrotic Activity in Human Lung Myofibroblasts. J Immunol 195:2852-60

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