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Rheumatologists urged to monitor for emerging fatal lung disease in systemic JIA
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.
“New data just recently published is giving us a new and concerning view of lung disease in systemic JIA,” 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.
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 outlined findings from the recent study by Saper and colleagues, published in Annals of Rheumatic Diseases, which demonstrated 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.
Stevens noted that these findings are consistent with another recent report by Schulert and colleagues, published in Arthritis & Rheumatology, which found a similar increase in pulmonary disease among children with systemic JIA, particularly in association with macrophage activation syndrome.
“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; data from the Saper study revealed a 5-year probability of survival of 42%.
“We are taking this very seriously and trying to learn more about it,” she said.
Saper and colleagues also reported 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.