Autoinflammatory diseases (AIFDs) involve overactive or dysregulated components of the innate immune system, leading to systemic inflammation that may result in serious damage to multiple organs. Over the past two decades, there have been dramatic developments in the field, including newly described AIFDs, increased understanding of pathogenic pathways of different AIFDs, and the introduction of new treatments for both monogenic and polygenic diseases. Despite these advances, a substantial portion of patients with AIFDs remain undiagnosed or experience diagnostic delay. Moreover, although several classes of drugs are available to manage patients with AIFDs, significant unmet needs persist in AIFD care.
In this CME-certified activity, AIFD experts will review the epidemiology and vital statistics on morbidity and mortality and the burden of disease in the United States resulting from delayed diagnosis. The activity will address the pathogenesis of AIFDs and present diagnostic criteria with and without genetic findings from the EULAR/SHARE guidelines. Recent recommendations for the management of select AIFDs will also be addressed as well as current research involving both established agents and those in development that hold promise in the treatment of autoinflammatory diseases.
Upon completion of this activity, participants should be able to:
1. Explain how new evidence surrounding the cellular pathophysiology of inflammasomes can inform currently available and emerging therapies designed to block pro-inflammatory signaling starting with interleukin-1.
2. Identify the clinical symptomatology of the most common monogenic & polygenic autoinflammatory diseases in the US that can render their diagnosis and targeted treatment including FMF, CAPS, SJIA, and Hidradenitis Suppurativa among others.
3. Discuss current research involving both established agents and those in development that target interleukin-1, with demonstrated efficacy for the treatment of autoinflammatory diseases in patients who fail to respond to prior treatment with TNFα-Inhibitors or other immunomodulators.
Scott Canna, MD
Assistant Professor, Pediatric Rheumatology
University of Pittsburgh School of Medicine
Scholar, Richard King Mellon Foundation Institute for Pediatric Research
University of Pittsburgh
Children’s Hospital of Pittsburgh of UPMC
Grant Schulert, MD, PhD
Assistant Professor of Pediatrics
Division of Rheumatology, Cincinnati Children’s Hospital
Department of Pediatrics, University of Cincinnati College of Medicine