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Children with GPA hospitalized more often than adult patients
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Children who have granulomatosis with polyangiitis are hospitalized more frequently, and are more vulnerable to hematologic complications, than nonelderly adults with the same condition, according to findings published in Arthritis & Rheumatology.
“Due to its rarity, much less is known about granulomatosis with polyangiitis (GPA) in children,” Sirada Panupattanapong, MD, of Washington University division of pediatric rheumatology, and colleagues wrote. “Studies of pediatric GPA have focused primarily on description of presenting clinical characteristics in small cohorts, including a female preponderance and higher prevalence of [ear-nose-throat] involvement when compared to adults. ... However, the data in these studies were incomplete and a better evaluation of outcomes in children is warranted.”
To characterize the epidemiology and outcomes of GPA in patients across the United States, and to compare the outcomes of pediatric and working-age adult patients, the researchers conducted a retrospective cohort study of data in the Truven Health Analytics MarketScan Commercial Claims and Encounters database.
Focusing on data from Jan. 1, 2006, to Dec. 31, 2014, Panupattanapong and colleagues identified 5,566 cases of GPA, including 214 children and 5,352 adults younger than 65 years. They then calculated the incidence and prevalence rates of pediatric and adult-onset GPA cases per million person-years.
According to the researchers, the incidence rate of pediatric GPA was 1.8 cases per million person-years, compared with 12.8 cases per million person-years for adult-onset cases. The researchers also noted a slight female preponderance in both the pediatric and working-age adult cohorts — 63% and 54% respectively. Although hospitalization rates were high in both groups, children with GPA visited the hospital more frequently (RR = 1.3; 95% CI, 1.1-1.4) than the adults. In addition, children with GPA demonstrated two to three times higher rates of leukopenia (RR = 2.6; 95% CI, 1.54.3), neutropenia (RR = 2.2; 95% CI, 1.24) and hypogammaglobulinemia (RR = 3.7; 95% CI, 2.6.4) than adult patients.
“Severe infections and hospitalizations were the most common adverse complications for children and adults with GPA,” Panupattanapong and colleagues wrote. “Pediatric patients experienced higher rates of hematologic complications and hospitalizations than their adult counterparts, whereas the rates of [end-stage renal disease], relapse and severe infection were comparable. Longer-term studies with large cohorts are still needed to better characterize the rare and long-term complications of GPA. Future work will investigate the role of different treatment strategies on these outcomes to better improve the quality of care of these patients.” – by Jason Laday
Disclosure: Panupattanapong reports no relevant financial disclosures. Please see the study for all other authors’ relevant financial disclosures.
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