Close
Enter your
text here

Kids With GPA Often Show Lung Signs — But Few See Pulmonologists

Kids With GPA Often Show Lung Signs — But Few See Pulmonologists 1024 683 Jessica Nye, PhD

Most patients with pediatric granulomatosis with polyangiitis (GPA) have respiratory manifestations, but fewer than half are evaluated by a pulmonologist within 6 months of diagnosis, finds a study published in Pediatric Pulmonology.  

“Pediatric GPA is a very rare autoimmune rheumatologic disorder that can have significant impact on the lungs. But nobody has really described the changes in the lungs over time,” says Katelyn Krivchenia, MD, a member of the Section of Pediatric Pulmonary Medicine at Nationwide Children’s Hospital, an associate professor at The Ohio State University College of Medicine and senior author of the study.  

Led by first author Shilpa Sridhar, MD, of The Ohio State University, this retrospective study assessed respiratory manifestations, contact with pulmonology and treatment modifications in 6-month intervals among patients (N=13) diagnosed with GPA at Nationwide Children’s between 2010 and 2021.   The patients had an average age of 14.8 years at diagnosis, 83% were girls and 17% had preexisting asthma.  

At diagnosis, 83% of patients had any respiratory symptom, 92% had an abnormal chest imaging and 50% had abnormal pulmonary functional testing (PFT). The most frequent respiratory signs were cough (50%), dyspnea (50%) and oxygen requirement (42%). The most common PFT abnormalities included FEV1/FVC ratio (38%), FEV1 (25%), FVC (25%) and TLC (25%).  

Despite these trends, a minority of patients (42%) had a pulmonary consultation within 6 months of diagnosis. In all 44 observational periods with pulmonary symptoms, 29% had pulmonology encounters and 50% did not include additional chest imaging or lung functional testing.  

“Not very many patients were actually seen by a pulmonologist in the first six months of their care, even up to a year,” says Dr. Krivchenia. “I am hoping to highlight through this work how important it is to have a pulmonary included early in care. Not every respiratory symptom may be related to the GPA, but testing and surveillance of the respiratory system may help the team guide therapy.”  

Longitudinally, most patients (67%) had persistent or recurrent respiratory symptoms of cough and/or dyspnea.  

The patients received induction treatment with systemic steroids (92%), cyclophosphamide (75%), rituximab (13%) and/or methotrexate (8%). In reaction to systemic symptoms, imaging outcomes, PFT abnormalities, laboratory abnormalities and/or adverse events, the maintenance treatments were amended to systemic steroids (83%), azathioprine (58%), methotrexate (42%), rituximab (25%), mycophenolate mofetil (25%), hydroxychloroquine (8%) and/or tacrolimus (8%). 

Dr. Krivchenia concludes, “Management of GPA is multidisciplinary, and pulmonologists should be part of that team, if only to help ensure cohesive care. At Nationwide Children’s we have a pulmonologist in the vasculitis clinic with rheumatology once a month, so they are available for these children who have multisystemic manifestations of their disease.”

 

 

Reference:

Sridhar S, Akoghlanian S, Krivchenia K. Respiratory manifestations of pediatric granulomatosis with polyangiitis: A 12‐year experience from a tertiary care facility. Pediatric Pulmonology. 2025;60(1):e27311. 

Image Credit: Adobe Stock

About the author

Jessica Nye, PhD, is a freelance science and medical writer based in Barcelona, Spain. She completed her BS in biology and chemistry and MS in evolutionary biology at Florida State University. Dr. Nye studied population genetics for her doctorate in biomedicine at University of Pompeu Fabra. She conducted her postdoctoral research on the inheritance of complex traits at the Autonomous University of Barcelona.

You might also like