The Clinical Core of this project will function to carefully characterize a cohort of subjects with scleroderma, drawn from two large referral centers, followed prospectively to link their clinical data, disease progression and severity with biologic mechanistic data.
The specific aims of this proposal are:
Aim 1 : To develop and maintain a clinical data repository of key clinical disease parameters in a prospectively followed cohort of subjects with scleroderma and match these clinical data with subject tissue and blood samples, which will be stored and maintained by the Clinical Core. The clinical core will also coordinate the collection of blood samples for preparation of RNA from peripheral blood mononuclear cells (PBMC) to be carried out by the microarray core. It will also coordinate the collection of skin biopsies for fixation and histological evaluation and for RNA preparation in coordination with the microarray core.
Aim 2 : To provide investigators in Projects 1, 2 and 3 with clinical data and statistical resources to perform analyses correlating gene array and biomarker data with matched clinical data collected longitudinally.

Public Health Relevance

This project proposes to identify markers and predictors of clinical subsets and/or complications of scleroderma such as pulmonary hypertension, progression of skin involvement and development of interstitial lung disease. This biomarker identification will facilitate understanding of the causes of the disease and provide opportunities for intervention.

Agency
National Institute of Health (NIH)
Institute
National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
Type
Specialized Center (P50)
Project #
5P50AR060780-04
Application #
8731065
Study Section
Special Emphasis Panel (ZAR1-MLB)
Project Start
Project End
Budget Start
2014-09-01
Budget End
2015-08-31
Support Year
4
Fiscal Year
2014
Total Cost
$450,090
Indirect Cost
$152,912
Name
Boston University
Department
Type
DUNS #
604483045
City
Boston
State
MA
Country
United States
Zip Code
02118
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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
Johnson, Michael E; Grassetti, Andrew V; Taroni, Jaclyn N et al. (2016) Stress granules and RNA processing bodies are novel autoantibody targets in systemic sclerosis. Arthritis Res Ther 18:27
Rice, Lisa M; Stifano, Giuseppina; Ziemek, Jessica et al. (2016) Local skin gene expression reflects both local and systemic skin disease in patients with systemic sclerosis. Rheumatology (Oxford) 55:377-9
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
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
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
Salazar, Gloria A; Assassi, Shervin; Wigley, Fredrick et al. (2015) Antinuclear antibody-negative systemic sclerosis. Semin Arthritis Rheum 44:680-6
Mathes, Allison L; Rice, Lisa; Affandi, Alsya J et al. (2015) CpGB DNA activates dermal macrophages and specifically recruits inflammatory monocytes into the skin. Exp Dermatol 24:133-9
Johnson, Michael E; Pioli, Patricia A; Whitfield, Michael L (2015) Gene expression profiling offers insights into the role of innate immune signaling in SSc. Semin Immunopathol 37:501-9

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