Pediatric-onset sarcoidosis may require long-term treatment, and can be associated with severe consequences in adulthood, according to a French study.
Of 52 adults with sarcoidosis as children, half were still being treated and four had uncontrolled disease, its researchers reported.
The study, “Child–Adult Transition in Sarcoidosis: A Series of 52 Patients,” was published in the Journal of Clinical Medicine.
Sarcoidosis shows striking differences in children versus adults. Pediatric sarcoidosis is a rare and mostly severe disease. Studies reported that the disease usually affects more than four organs in almost half of pediatric patients, compared with 7% of adults.
Children with the disease experience symptoms such as fever, weight loss, and sweats more frequently than adults.
Little is known, however, about how pediatric sarcoidosis evolves as the child ages, as both the frequency and duration of follow-up visits varies widely.
To assemble a picture of how pediatric sarcoidosis evolves over the long term, researchers in France analyzed the medical records of adults whose sarcoidosis began as children (before the age of 16) and who had at least three years of follow-up information. All were followed at French specialized centers.
In total, a group of 52 patients were analyzed. Thirty-four were enrolled prospectively in childhood, and 18 retrospectively in adulthood. Their average age at diagnosis was 12.03 years, and their median duration of follow-up was 11.48 years. Twenty-nine patients (56%) were female, and 71.15% had Sub-Saharan or Afro-Caribbean ancestry (of note, sarcoidosis is more common among people of African and northern European descent).
In general, relapses occurred more frequently when treatment was lowered or interrupted. Eleven of the patients (21%) had severe, life-threatening sarcoidosis in adulthood.
Among the 34 patients enrolled in childhood, 18 were asymptomatic by the end of their pediatric follow-up period, with no relapses during adult follow-ups. Two were stable at the end of their pediatric follow-ups, but experienced relapses as adults.
Among the 18 patients retrospectively enrolled as adults, three were asymptomatic at the end of their childhood follow-ups, with no relapses reported as adults. Four were stable by the end of their pediatric follow-ups, but experienced relapses as adults.
One in five stable patients experienced relapses as adults, and only one (3.8%) of the patients under treatment at the time of their pediatric-to-adult transition had no later relapse.
“Taking into account the whole series, at the end of pediatric follow up, half of the patients were stable (no activity of the disease while without treatment); the other 26 (50%) patients still required a treatment,” the researchers wrote.
At the last adult evaluation, 28 patients (53.8% of the cohort) were still under treatment. Among these, four had uncontrolled disease despite treatment. No deaths were recorded during the study, but one patient required a lung transplant at age 49.
The team also analyzed the number of organs affected, and found it to be significantly lower in adulthood. The number of affected organs in childhood appeared to best predict the risk of a patient’s condition becoming severe as an adult. Among these severe cases, the lungs were the organ most associated with severity.
The oldest patients in the study — ages 49 and 55 — had the most severe disease and the longest period of progression.
Most patients (94%) were given corticosteroid therapy over an average of five years. Despite a relatively high use of corticosteroids — described as an “impressive cumulative dose” by the team — few adverse events were recorded, and only two patients had a severe event (a bacterial septic shock and community-based pneumonia requiring hospitalization).
Based on the results, the team concluded that “pediatric-onset sarcoidosis is a rarely reported condition that often evolves toward a chronic disease in adulthood, with almost half of patients requiring long-term treatment.”
“Regarding the initial severity of the disease, the risk of long-term relapse and the potential adverse effects of prolonged treatments, a long-term follow-up is highly recommended in patients with pediatric-onset sarcoidosis,” the researchers added, noting that the “severity and morbidity of this disease should not be underestimated, and argue for a life-long follow up of these patients.”