Johns Hopkins Scleroderma Center of Excellence

Fredrick Wigley, MD
Johns Hopkins University School of Medicine

Project Overview

Dr. Wigley: The Johns Hopkins Scleroderma Center of Excellence now has seven full-time faculty members who evaluate and manage scleroderma patients referred to us from all over the world. Drs. Chris Mecoli and Julie Paik both also see patients with muscle diseases. All the faculty work as clinical investigators. Members of the faculty take full advantage of the expertise at Johns Hopkins (JH) by utilizing superb specialists in other areas of medicine (skin, lung, heart, gastrointestinal tract, psychological, endocrine) and surgery (plastics, vascular, orthopedic) to help manage the complex care our patients require. We received about 350 new patient requests in 2018. The Center now has over 3,800 scleroderma patients in our research database; we evaluate and manage 10-11 new patients and 60-70 return scleroderma patients each operational week.  This clinical practice remains fully integrated with our research and educational programs.

The Scleroderma Center has built a world-renowned framework for characterizing the breadth of scleroderma phenotypes, investigating underlying disease mechanisms and testing novel therapies for scleroderma. The cornerstone of this approach involves providing comprehensive, longitudinal patient care to scleroderma patients and, at the same time, prospectively collecting and storing clinical data and biological samples from patients. Coupling this exceptional data and sample resource to the discovery engine at Johns Hopkins has resulted in outstanding productivity.

Each of our faculty has a defined research focus and they interact to support the overall research activity. Dr. Fredrick Wigley provides leadership and direction to the program. Dr. Laura Hummers continues to act as the JH site Principal Investigator (PI) for multiple clinical trials with pharmaceutical companies and investigator-initiated studies. Dr. Ami Shah recently discovered that cancer (many types) can be associated with and likely trigger the onset of scleroderma. Building on her work funded in part by the SRF, she now has NIH support to further investigate the interaction of cancer, autoimmunity, and scleroderma.  Drs. Hummers and Shah are the PIs for the CONQUER registry, a multicenter effort to develop a large cohort of scleroderma patients and clinical samples for collaborative research. Dr. Zsuzsanna McMahan is leading research to better understand the impact and cause of gastrointestinal disease among scleroderma patients. Dr. Julie Paik is classifying and studying the pathogenesis of scleroderma-related muscle disease. Dr. Nadia Morgan is active in the Genome Research in African American Scleroderma Patients (GRASP) Project; a multicenter interactive program to understand why African Americans suffer a greater burden of scleroderma disease. Dr. Christopher Mecoli is investigating biomarkers that can better define disease activity and predict outcomes including treatment responses.

We continue several studies with Drs. Antony Rosen and Livia Casciola-Rosen and others to better understand how the immune system initiates and propagates the disease process as well as studies with Dr. Hal Dietz’s lab investigating mechanisms of regulating tissue fibrosis. All our faculty continue to collaborate with Dr. Scott Zeger, Professor of Biostatistics, with a goal of better predicting clinical outcomes by using our rich database and trajectory modeling. Our Center’s research program continues to have exceptional momentum and is making major progress in understanding the disease process and novel treatments for this disease.  The Educational Program also continues to be another major priority of our Center, driven by a faculty known for their expertise both as clinical scientists and educators.

Role of the Scleroderma Research Foundation

The best way to understand how the SRF has played a key role in our program is to see the incredible growth of the Johns Hopkins Scleroderma Center. Several of our critical assets, such as our clinical database and biorepository, as well as the physicians, fellows, clinical coordinators and database managers who are so critical to our research efforts, have received long-standing support from the SRF. Our ability to conceive of and execute cutting-edge research depends on these assets and critical personnel. Further, the SRF has invested in projects that stem from the nuanced clinical observation and longitudinal care that are prized at our Center. Our program would not have the resources to fully operate without the support of the SRF. In fact, the funds provided by the SRF have made it possible for our faculty to get additional funding from the NIH and Department of Defense.

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