A disease that has no cause or cure, sarcoidosis affects people of all ages throughout the world. Once considered a rare disease, the inflammatory condition now affects about 40 in every 100,000 African Americans in the U.S. and about five in every 100,000 white people. It occurs in all races and in men and women, but is most common among African American females ages 20 to 40.   

The disease most commonly affects the lungs, but can involve almost any organ system including the skin, eyes, joints and heart. Cardiac involvement, which occurs in up to 25% of patients with sarcoidosis in other organs, can lead to life-threatening heart rhythm problems and heart failure.

“The field of cardiac sarcoidosis, and sarcoidosis in general, really needs new mechanistically driven therapies,” said Jordana Kron, M.D., an associate professor in the Virginia Commonwealth University School of Medicine and a cardiologist at the VCU Health Pauley Heart Center. 

This month, which is National Sarcoidosis Awareness Month, Kron was awarded a $50,000 Pauley Pilot Research Grant to investigate a new treatment protocol for cardiac sarcoidosis. The 15-month grant will be used to evaluate the efficacy and safety of using an interleukin-1 blockade to treat patients who present with cardiac sarcoidosis. The study is the first of its kind to explore the new treatment paradigm.

“A new, safe and effective treatment could be life-altering for patients with cardiac sarcoidosis. It may also open the door for new therapies for cardiac sarcoidosis and other inflammatory heart diseases in the future,” said Kron, a translational science scholar at the VCUC. Kenneth and Dianne Wright Center for Clinical and Translational Research.

Interleukin-1 is a protein whose presence indicates inflammation in the body. Kron’s research aims to evaluate if using medication to block the protein will help treat cardiac sarcoidosis.

“Corticosteroids are the most commonly used treatment for sarcoidosis, but there is little data showing their efficacy and they have significant side effects,” Kron said. “Interleukin-1 is the prototypical cytokine that plays a role in most inflammatory processes. Blockade of the protein has been shown to be effective in many types of heart disease.”

While cardiac sarcoidosis is the focus of Kron’s research, she is optimistic that— if shown to be effective — the blockade could be used to treat other forms of heart disease.

Tackling cardiac sarcoidosis

VCU has led clinical and translational research of cardiac sarcoidosis for nearly a decade and VCU Health is home to the Multidisciplinary Sarcoidosis Clinic, the only center of its kind in Virginia. At the clinic, located at the VCU Health Stony Point Campus, patients are able to meet with specialists in pulmonology, cardiology, electrophysiology and rheumatology during a single appointment.

In 2011, VCU researchers teamed with researchers at the University of Michigan and the University of Colorado to start the international Cardiac Sarcoidosis Consortium. Kron is a founding member. The consortium is a prospective, multicenter registry that tracks cardiac sarcoidosis patients worldwide. It includes demographic, clinical, medication and imaging data from more than 25 contributing centers in the U.S., Europe and Asia.

Kron attributes much of the headway she has made in researching sarcoidosis to support from the Wright Center, Virginia’s first institution to receive a Clinical and Translational Science Award from the National Institutes of Health. Antonio Abbate, M.D., Ph.D., associate director of the Wright Center, has served as a mentor and co-investigator on Kron’s research. Abbate, a cardiology professor at the VCU School of Medicine and a cardiologist at the Pauley Heart Center, also will share his expertise on cardiac inflammation and interleukin-1 blockers throughout the study. F. Gerard Moeller, M.D., director of the Wright Center, and Patrick Nana-Sinkam, M.D., the center’s KL2 program co-director, provided mentorship, support and feedback on grant writing for the project as well.

“Support from the Wright Center has enabled me to build on existing relationships and create new collaborations to help advance our understanding and treatment of this complex disease,” Kron said.

Kenneth Ellenbogen, M.D., chair of the Pauley Heart Center’s Division of Cardiology, also provided significant support for Kron’s research.

“I would not be where I am today without Dr. Ellenbogen’s mentorship,” she said. “He has played an invaluable role in my research career.”

Gathering critical data

Kron, who last October was awarded an endowment fund through the Wright Center to further support her research, is joined in the study by Jennifer Jordan, Ph.D., an assistant professor in the VCU College of Engineering Department of Biomedical Engineering and director of the Cardiovascular MRI Core Lab at the Pauley Heart Center. Jordan’s research focuses on translational and clinical cardiovascular MRI techniques and cardiac magnetic resonance tissue characterization in patients who have received chemotherapy for breast cancer. For this study, she will be using her expertise in cardiac magnetic resonance to help assess inflammation in cardiac sarcoidosis and evaluate responses to treatment. 

The Pauley Pilot Research Grant Program, which is made possible entirely by philanthropy, supports early stage research by physicians and scientists working to advance heart health. The program allows investigators to test novel ideas and gather enough data to apply for major research grants from institutions such as the NIH.

Kron plans to submit a proposal for external funding by February 2021.

“The results of this research will help me gather critical data to inform applications for larger grants later,” she said.