Respiratory manifestations common, recurrent in pediatric GPA
Care teams should collaborate early, researchers say
Most children with granulomatosis with polyangiitis (GPA) have respiratory manifestations including cough and shortness of breath at diagnosis, and these frequently persist or relapse, according to a study in the U.S. involving 13 pediatric patients.
Lung abnormalities were detected in nearly all children with available imaging data at diagnosis regardless of respiratory symptoms.
“Early collaboration with pediatric pulmonology in the care of GPA patients may allow rheumatology teams to efficiently evaluate recurrent symptoms and address concomitant lung disease,” the researchers wrote. “On a broader scale, the input of pulmonologists in guideline creation could help standardize recommendations for diagnostic modalities and management of pulmonary manifestations in GPA patients,” they added.
The study, “Respiratory manifestations of pediatric granulomatosis with polyangiitis: A 12-year experience from a tertiary care facility,” was published in Pediatric Pulmonology.
GPA is a type of ANCA-associated vasculitis (AAV), a group of autoimmune diseases characterized by inflammation and damage to small blood vessels that are commonly caused by self-reactive antibodies called ANCAs. This common AAV type is usually characterized by symptoms affecting the respiratory tract and the kidneys.
Cough, chest pain among symptoms
Respiratory problems may manifest as symptoms including cough, coughing up blood, and sharp chest pain, and abnormal results on lung function tests and lung imaging scans.
Previous studies in children with GPA, which account for up to 7% of all GPA cases, have shown that respiratory symptoms are also common, affecting 44% to 79% of pediatric patients.
While “most prior research has described the prevalence of respiratory manifestations at the time of diagnosis,” no U.S. study has described the course of these manifestations in pediatric GPA patients over time, the researchers wrote.
To fill this knowledge gap, a trio of physicians at the Ohio State University retrospectively analyzed medical data from children with GPA who were followed at the Nationwide Children’s Hospital from January 2010 to December 2021.
Of the 13 children with a verified GPA diagnosis, all but one developed respiratory manifestation and were included in the analysis. These 12 children (83.3% girls) had a mean age of 14.8.
At diagnosis, all but two (83.3%) showed respiratory signs or symptoms, most commonly cough and shortness of breath (50% each), followed by need for oxygen supplementation (41.7%) and noisy breathing (8.3%).
All but one child (91.7%) had abnormalities in chest imaging scans at diagnosis, suggesting that imaging abnormalities are nearly universal in pediatric GPA patients, regardless of the presence of respiratory manifestations.
At diagnosis, children had an average of about two symptoms and two abnormalities in imaging tests.
Persistent symptoms
During the study period, all 11 children who underwent chest CT scan showed abnormal results, with the most frequent being lung nodules (55%), ground glass opacities (55%), and swollen lymph nodes in the chest (45%). Ground glass opacities are white, diffuse regions resulting from causes including fluid, airway collapse, and tissue scarring.
Six of the 10 children who completed lung function tests during the study exhibited abnormalities, which most commonly suggested airway obstruction.
Other lung tests performed at diagnosis included lung biopsy and bronchoscopy, which looks into the airways through a thin tube inserted in the nose or mouth.
In the six months after diagnosis, about two-thirds of the children (66.7%) experienced persistent or recurring respiratory symptoms, including cough and shortness of breath at rest and/or with exercise. Still, fewer than half (41.7%) were seen by a pulmonologist.
Of the 44 six-month observation periods during which children had respiratory symptoms, 12 (29%) involved assessment by a pulmonologist and half of these occurred in the first six months of care. Three-quarters of the 12 were hospitalized within six months of diagnosis.
In about three-quarters of the observation periods marked by respiratory manifestations, children also showed whole-body disease involvement, including low levels of red blood cells, high levels of immune cells, and signs of inflammation and kidney damage.
For treatment, most children received induction therapy with corticosteroids and/or cyclophosphamide (sold as Cytoxan, among others), and maintenance treatment with azathioprine and/or methotrexate. Five children were treated with rituximab (sold as Rituxan, among others), either for induction or maintenance therapy.
Despite the small number of patients included, and lack of comprehensive follow-up data for some of them, the study “highlights that pulmonary manifestations in pediatric GPA are common and recurrent,” the researchers wrote. “Specific guidance regarding diagnostic evaluation and long term follow up of pediatric GPA is warranted,” the trio wrote, adding that “chest imaging and [pulmonary function tests] should be obtained in all these patients.”
“Pulmonology involvement may facilitate appropriate testing in the setting of symptomatic recurrence and help with management of pre‐existing or newly recognized lung disease outside of the rheumatologist’s scope of practice,” the researchers concluded.
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