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Biologic use linked to fewer flares, higher rates of inactive disease in childhood Takayasu arthritis
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Children with Takayasu arthritis who were treated with biologic therapies exhibited significantly less flares and attained inactive disease status more often than children who received non-biologic treatments.
The researchers, who published their findings in Arthritis Research & Therapy, also revealed that the disease carries a high burden, with 48% of patients experiencing flares and 7% dying within 6 months of diagnosis.
“Takayasu arthritis (TAK) is a rare but potentially fatal disease with little published data,” Rae S. M. Yeung MD, PhD, FRCPC, professor of pediatrics, immunology and medical science at the University of Toronto, Ontario, told Healio Rheumatology. “Childhood TAK remains a horrible disease with dramatic morbidity and early mortality, with half of all children experiencing disease flares and 7% dying from their disease.”
“Childhood TAK is very heterogeneous, with a highly variable clinical phenotype depending on location and extent of vessel involvement,” Yeung said. “Unfortunately, no clinical variable is predictive of disease course or outcome.”
To analyze the presenting symptoms, course and outcomes of children with TAK, and to compare the efficacy of various treatments and identify high-risk factors for adverse events, the researchers conducted a single-center cohort study on consecutive children who fulfilled the criteria for the disease between 1986 and 2015. Twenty-seven children were included, of which 74% were girls, with a median age of 12.4 years.
The researchers documented clinical phenotypes, laboratory markers, imaging features, disease course and treatment. They assessed disease activity using the Pediatric Vasculitis Disease Activity Score at each visit. Disease flares were defined as new symptoms and/or increased inflammatory markers requiring increased therapy and/or new angiographic lesions, or death. Aeschlimann and colleagues used logistic regression to test relevant variables for flare, and Kaplan-Meier analyses to compare treatments.
According to the researchers, 15% of patients were treated with corticosteroids alone, 37% with corticosteroids plus methotrexate, 19% with cyclophosphamide and 11% with a biologic agent. Adverse outcomes were documented in 52% of children, including two who died within 6 months of diagnosis, and 13 who experienced disease flares. The rate of 2-year flare-free survival was 80% with biologic treatments, compared with 43% in nonbiologic treatments (P=0.03). At last follow-up, biologic treatments resulted in significantly higher rates of inactive disease (P=0.02).
“Childhood TAK is a devastating disease, lacking good clinical predictors of those with high-risk of poor outcome,” Yeung said. “Biologic agents targeting TNFa and IL-6 result in significantly better outcome and may be important considerations in our therapeutic arsenal. Collaborative studies in this rare disease are urgently needed to address the underlying pathobiology, identify biologic predictors of outcome, and the optimal therapeutic agents to improve outcomes for affected children and their families.” – by Jason Laday
Disclosure: The authors report no relevant financial disclosures.
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