Adult-onset Still’s disease (AOSD) is a rare autoinflammatory disease driven by the innate immune system leading to clinical manifestations of fever, rash, and arthritis. Additionally, AOSD patients can present with severe complications at onset including macrophage activation syndrome (MAS) and lung disease. Because large prospective cohorts of AOSD patients are lacking, research into long-term clinical outcomes and multimorbidity of this disease is limited.
Aleksander Lenert, MD, MS, FRCPC, clinical associate professor in the Division of Immunology, is a recent arrival from the University of Kentucky. Before his departure, he and some colleagues there and at NIH’s National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) performed an observational cohort study using administrative claims from a 2009–2015 Truven MarketScan database to identify clinical characteristics, complications, and treatment patterns in AOSD. Using the database, Lenert aimed to identify comorbidities in a large US-based AOSD cohort compared with matched controls. The study was published in last month’s issue of Oxford University Press’ Rheumatology.
“I’m very excited to share our study’s findings from this US-based AOSD cohort,” Lenert said. “We would like to build on these findings, especially to elucidate the course and outcome of severe lung disease in the era of biological therapies.”
In their study, Lenert and his colleagues identified severe AOSD-related complications, including MAS in 4.7% and acute respiratory distress syndrome in 12.3%. Treatments commonly identified were systemic glucocorticoids (62.2%), methotrexate (51%) and anakinra (24.5%). Compared with matched controls, serious infections were significantly increased, while hyperlipidemia and obesity were significantly decreased in AOSD patients.
“I believe that we can utilize ‘big data’ from large administrative claims and linked administrative-EMR [electronic medical records] databases to perform robust observational, clinical outcomes and comparative effectiveness studies in rare rheumatic diseases,” Lenert said. “Ultimately, our goal is to improve the understanding of the clinical landscape of AOSD and related autoinflammatory syndromes, and potentially lead to improved treatment strategies for our patients.”
POSTSCRIPT: Just after publication, we learned that Lenert will receive a one-year, $10,000 Innovation Research Grant from the Carver College of Medicine in support of a radiological study of salivary glands to improve diagnosis of seronegative Sjögren’s syndrome.