Researchers Need to Better Understand Differences in Eosinophilic Granulomatosis with Polyangiitis Symptoms in Children and Adults, Study Suggests

Children with eosinophilic granulomatosis with polyangiitis (EGPA) have specific disease symptoms that differ from those seen in adults with the disease, a French study shows.

Pediatricians should be aware of these differences and the various disease symptoms for a correct and timely diagnosis of EGPA in children, researchers said.

The study, “Eosinophilic granulomatosis with polyangiitis in children: Data from the French RespiRare® cohort,” was published in the journal Pediatric Pulmonology.

Eosinophilic granulomatosis with polyangiitis is a rare condition that causes inflammation of small- and medium-sized blood vessels.

The disease belongs to the classification of small vessel anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and may affect multiple organs, including the lungs, the gastrointestinal tract, the skin, the heart, and the nervous system.

But in children, the disease is rare and a general lack of understanding exists about how EGPA manifests in these patients.

Researchers in France set out to examine a cohort of children with EGPA and compare their symptoms with those seen in adults.

Pediatric patients were part of a French database called RespiRare, which included 33 French medical centers. Researchers also examined data from 383 adult patients registered in the French Vasculitis Study Group.

The study included 14 children and adolescents diagnosed between 1980 and 2012. Patients had been diagnosed at a median age of 12.3 years and were followed for a median of 58.5 months.

Using a standardized questionnaire directed at the coordinators of each center, researchers collected a series of data, including clinical features at the time of diagnosis, prognostic score, treatment, and outcomes for each patient.

Researchers noted that children were diagnosed faster after the onset of asthma than adult patients. And while adults had more neurological and renal symptoms, children had significantly more symptoms in the ears, nose, and throat, as well as the heart, skin, and gastrointestinal tract.

Among the 14 children, only four (30.7 percent) were positive for ANCA autoantibodies. These children had more neurological symptoms than children who were negative for the antibodies.

Sustained elevations of eosinophil, a type of immune cell, were seen in six patients. This complication can lead to organ damage, researchers noted.

All of the children received treatment with corticosteroids with or without immunosuppressants. However, nine children, or 64 percent, relapsed at a mean time of eight months after diagnosis. While this was higher than the relapse rate seen in adults, no significant difference was seen in mortality rates.

“Pediatricians should be familiar with the specific presentation and spectrum of organ damage of EGPA in childhood in order not to misdiagnose a refractory asthma with extrapulmonary symptoms,” researchers wrote.

“The recent classification described by the European Respiratory Society (ERS) and ERS/GERM“O”P seems to be very poorly applicable to children. Future pediatric studies based on a larger cohort are necessary to establish pediatric diagnostic criteria for EGPA,” the researchers concluded.