Despite the fact that pulmonary artery hypertension (PAH), interstitial lung disease (ILD) and alveolar proteinosis (AP), are increasingly being seen among patients with Systemic Juvenile Idiopathic Arthritis (sJIA), doctors apparently are not recognizing, and thus not treating, these secondary conditions. This is leading to dire results among patients, including death.
A large international team, headed by Yokiko Kimura of the Hackensack University Medical Center in New Jersey, culled an electronic listserv to find patients who had both sJIA and/or any of the secondary health issues listed above, and then compared this list to a sampling of children found in the registry of the US-Canadian Childhood Arthritis and Rheumatology Research Alliance (CARRA). The majority of the children found on the listserv was female, had taken at least one drug such as tocilizumab, infliximab(Drug information on infliximab), or corticosteroids, and had more systemic features.
Sixty percent of the patients from the listserv group showed macrophage activation syndrome (MAS) during pulmonary diagnosis, while 80% developed it over the course of their disease. All of them showed at least one of the secondary conditions. Sixty-eight percent were taking or had recently discontinued a biologic agent at the time their pulmonary complications developed, and nearly half were taking an anti-IL1 therapy.
Ultimately, 68% of people on the listserv died of one of these pulmonary disorders approximately 9 months after they were diagnosed. It is not clear the extent to which the disorders are a result of lack of disease control or of exposure to the medication. In either case, screening for PAH, ILD and AP should be considered in all patients with significant disease activity, say the researchers.
The study was reported as Abstract #762 at the American College of Rheumatology meeting in Washington DC and published in Arthritis Care and Research.
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