The Johns Hopkins Scleroderma Center of Excellence

Fredrick M. Wigley, MD
Johns Hopkins University School of Medicine

The Johns Hopkins Scleroderma Center focuses on clinical and translational research in the context of comprehensive patient care. The Center was launched in 1990 with seed funding from the SRF and is now one of the largest and most highly regarded scleroderma centers in America. 

Project Summary:

Dr. Wigley: The Johns Hopkins Scleroderma Center of Excellence now has four full-time faculty members and one part-time faculty member who evaluate and manage scleroderma patients referred to us from all over the world. Additionally, two of the Center’s outstanding fellows, Dr. Nadia Morgan and Dr. Chris Mecoli, will join the Center’s faculty as clinical-investigators. Members of the faculty continue to interact daily, discussing complex cases. We also 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 450 new patient requests in 2016. The Center now has over 3,500 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.

Investigators in and affiliated with our Center recently discovered that cancer (many types) can be associated with and likely trigger the onset of scleroderma and together we are working to determine exactly how this happens. The work on the cancer-scleroderma connection brings together outstanding investigators across oncology, cancer genetics, and immunology. This year Dr. Ami Shah has worked with Dr. Tak Igusa, a civil engineer and leader in the Johns Hopkins Systems Institute, to study the prevalence of cancer among our patients with scleroderma compared to the general population without scleroderma. They confirmed an increased risk for cancer among patients with the anti-RNA polymerase III antibody and a decreased risk of cancer among patients who are anti-centromere positive. This work now guides physicians in understanding which patients with scleroderma have a unique risk of cancer and need to be carefully screened. Additionally, we believe that treating the cancer will have a beneficial impact on the scleroderma disease process and, in addition, managing the immune system may help control cancer.

We continue several studies with Drs. Antony Rosen and Livia Casiola-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.

We continue to provide leadership to a multi-center program called GRASP. We also continue our collaborative work with Drs. Suchi Saria and Scott Zeger working on the Trajectory Modeling project.

Dr. Zsuzsanna McMahan has discovered novel autoantibodies that associate with vascular disease and she is working on the gastrointestinal manifestations of scleroderma. Dr. Laura Hummers continues to act as the JH site Principle Investigator for multiple clinical trials with pharmaceutical companies and investigator-initiated studies.

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 effort continues to be another major priority of our Center, driven by a faculty known for their expertise both as clinical scientists and educators.

Dr. Wigley is one of the editors of the recently published textbook Scleroderma: from Pathogenesis to Comprehensive Management.

Dr. Wigley on Why Research Matters:

“Without research and efforts to work toward new discoveries, we would be forever stuck in traditional modes of patient care. We know now that past research efforts have taught us lessons that have improved not only our understanding of scleroderma and the disease process, but have also led us to new and better ways to treat patients. We are making a difference right now due to our improved methods for treating the disease. Research not only defines the process, it forces the caregiver to move from failed methods to exciting and novel therapies.”

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