Young age, adverse drug reactions may signal lung disease in juvenile idiopathic arthritis
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SCOTTSDALE, Ariz. — The prevalence of systematic-onset juvenile idiopathic arthritis-related lung disease appears to be on the rise, and being aware of clinical manifestations is important for catching the disease early, a speaker said.
“This caught my eye, that we may be dealing with a new disease or at least a disease that has become much more frequent, I think that is sort of a worrisome kind of thing, but it is also something that we have to try to jump into to get our head around,” R. John Looney, MD, of the allergy, immunology and rheumatology division at the University of Rochester, told attendees at the Basic and Clinical Immunology for the Busy Clinician symposium. “In kids with systemic onset-JIA, they can get lung disease.”
According to Looney, the number of cases of lung disease each year increased at a specific center from 2011 to now. In the same vein, the number lung biopsies revealing pulmonary alveolar proteinosis or endogenous lipoid pneumonia also increased at the same center, he said.
When examining a patient with JIA, rheumatologists should keep certain warning signs in mind to catch lung disease manifestations, Looney said. Specifically, he added that rheumatologists should be suspicious of atypical rashes or clubbing.
“This is not a rash that is coming and going, this is coming and it is staying,” Looney said. “You can get clubbing of the fingers or the toes of these young infants.”
These manifestations should come across as “striking” and “unusual” in patients with systemic-onset JIA (SOJIA), according to Looney.
Additionally, physicians should be aware of some of the risk factors associated with an increased likelihood of disease in patients with SOJIA. These factors include being younger than 2 years of age, experiencing one or more adverse drug reactions to tocilizumab (Actemra, Genentech) and a history of macrophage activation syndrome or severe disease.
“The risk if you’re younger than 2 is markedly increased, as well as if you have had a history of an adverse drug reaction or if you have had a history of macrophage activation syndrome,” Looney said. “The concern here is that this systemic-onset JIA with lung disease is very lethal. This is obviously something that we have got to figure out.”
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Looney RJ. Immunologic insights into lung disease in SOJIA and adult-onset Still’s disease. Presented at Basic and Clinical Immunology for the Busy Clinician, Feb. 25-26, 2023; Scottsdale, Arizona (hybrid meeting).
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