Although childhood-onset Takayasu arteritis has a higher frequency of aortic and renal involvement compared with the adult-onset form of the disease, relapse and disease burden remain high among patients in both groups, according to findings published in Arthritis & Rheumatology.
“Differences in disease expression of [Takayasu arteritis (TAK)], including gender ratio, vascular involvement and disease course have been reported between children and adults,” Florence A. Aeschlimann, MD, MPH, of the Hospital for Sick Children at the University of Toronto, and colleague wrote. “Childhood-onset and adult-onset TAK patients have been compared in China and Brazil. Given that geographic or ethnic variations in disease presentation have been reported, we investigated childhood-onset and adult-onset TAK cohorts in a North American population.”
To compare the characteristics, the efficacy and safety of treatment regimens, and the outcomes of adult- and childhood-onset TAK, the researchers conducted a retrospective cohort study of 29 juveniles and 48 adults from four centers in Ontario, Canada. The researchers focused on patients who have lived with childhoodonset TAK since 1986 and adultonset TAK since 1988. All participants were followed until 2015.
Although childhood-onset TAK has a higher frequency of aortic and renal involvement compared with the adult-onset form of the disease, relapse and disease burden remain high among patients in both groups, according to findings.
Aeschlimann and colleagues collected data on all participants, including demographics, past medical history, clinical presenting features of TAK, investigations, treatment and adverse outcomes, which were defined as relapse, death or complications. Disease activity and damage scores were later completed by the researchers,
According to the researchers, patients with childhood-onset TAK had a 76% female predominance, compared with 100% among those who developed the disease as an adult (P < .01). Those with childhood-onset TAK also experienced a shorter delay in receiving a diagnosis, with a median wait time of 6 months, compared with a 12.2-month delay for adults. In addition, patients with childhood-onset TAK demonstrated significantly more aortic and renal artery involvement, with a higher frequency of arterial hypertension, the researchers wrote. However, relapses in the first year following diagnosis were common among both groups — 39% in those with childhood-onset TAK and 28% for adults. Among the participants, two children and no adults died.
“This is the first study describing the presentation and disease course of childhood- and adult-onset TAK in North America,” Aeschlimann and colleagues wrote. “Relapses and disease burden were high in both groups corroborating the need for careful monitoring of disease activity and aggressive therapeutic management in order to prevent mortality and long-term morbidity. The optimal methods for monitoring and treating TAK patients yet require further study TAK in North America.” – by Jason Laday
Disclosure: Aeschliman reports no relevant financial disclosures. Please see the study for all other authors’ relevant financial disclosures.