Rheumatologists urged to monitor for emerging fatal lung disease in systemic JIA - Healio

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Rheumatologists urged to monitor for emerging fatal lung disease in systemic JIA - Healio Posted: 20 Feb 2020 01:27 PM PST Anne M. Stevens MAUI, Hawaii — Lung complications in systemic juvenile idiopathic arthritis are gaining more attention due to increased incidence, according to a presenter at the 2020 Rheumatology Winter Clinical Symposium. "We have some really interesting new data," Anne M. Stevens, MD, PhD, of the department of rheumatology at Seattle Children's Hospital and affiliate clinical professor in the department of pediatrics at the University of Washington, said during her presentation. Stevens raised several key questions about lung disease in JIA, including whether it is similar to or as common as lung disease in rheumatoid arthritis, whether methotrexate contributes and whether there is a genetic basis. "We think that any of these diseases with an earlier onset may have a genetic basis," she said, adding that several data sets have ruled out methotrexate as a potential etiological factor.   Lung complications in systemic JIA are gaining more attention due to increased incidence, according to Stevens. Source: Adobe Stevens provided an overview of the features of systemic JIA as reported in recent publications, including lymphadenopathy and serositis, along with fever, arthritis, rash and hepato-splenomegaly. "I do not see lung disease on here," she said. "Why then, are we starting to see this increase in reports of lung disease in systemic JIA?" Stevens asked. She reported on a study that showed that between 1970 and 1999, there were 1.3 cases of lung disease in systemic JIA for every 5-year increment. That figure increased to five cases for 2000-2004, 25 for 2005-2009, 31 for 2010-2014, and 74 for 2015-2019. "You can see it is marching up as the years roll on," Stevens said. Stevens stressed that these reports are accompanied by data showing that lung disease can significantly impact survival for these patients. Another study Stevens shared revealed a 5-year survival rate of 42%. "We are taking this very seriously and trying to learn more about it," she said. Stevens described a third study that showed that pulmonary arterial hypertension (PAH) occurred in 64% of 25 cases of systemic JIA. In the researchers' follow-up study, which included 45 additional patients with true systemic JIA, they found that those with lung involvement were considerably younger than other systemic JIA patients, and were more likely to have trisomy 21. "These are two interesting risk factors that maybe give us a clue," Stevens said. Other unusual features reported in these patients with lung involvement included pruritic rash, a reaction to tocilizumab, peripheral eosinophilia, acute clubbing with bright red fingers, lymphopenia, and abdominal pain. Stevens then pointed out a paradoxical feature that can be seen in these children. "The respiratory symptoms are actually very subtle," she said. "Maybe a mild cough, but what kid does not have a cold or a cough?" This brought Stevens to a key point for rheumatologists to consider. "Most of the lung disease in children with JIA is asymptomatic until late," she said, and encouraged clinicians to monitor patients with any of the potential features. – by Rob Volansky Reference: Stevens A. Lung Disease in Pediatric Rheumatology. Presented at RWCS Annual Meeting; Feb. 12-15, 2020; Maui, Hawaii. Disclosures: Stevens reports having a PD-L1 patent with Quest Diagnostics; research collaborations with Kineta and Seattle Genetics; fellowship support from Pfizer; and being an employee of Janssen Pharmaceuticals.

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