RESUMEN
Eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome) is classified as an antineutrophil cytoplasmic antibody (ANCA)-associated small vessel vasculitis. It is a multisystem disorder and can affect every organ system. EGPA is a rare disease, with an estimated prevalence of 1/70,000-100,000 in Europe. As its onset usually occurs in adulthood, data from pediatric patients are limited. We present here a very rare practical EGPA clinical case involving a pediatric patient. Presently, data on mepolizumab usage in pediatric patients are limited, with only a few case reports published.
Asunto(s)
Síndrome de Churg-Strauss , Granulomatosis con Poliangitis , Humanos , Niño , Síndrome de Churg-Strauss/complicaciones , Síndrome de Churg-Strauss/tratamiento farmacológico , Síndrome de Churg-Strauss/epidemiología , Granulomatosis con Poliangitis/complicaciones , Granulomatosis con Poliangitis/tratamiento farmacológico , Anticuerpos Monoclonales Humanizados/uso terapéutico , Glucocorticoides/uso terapéutico , Anticuerpos Anticitoplasma de NeutrófilosRESUMEN
We aimed to evaluate the efficacy and safety of fluticasone propionate (FP) in children aged 12-47 months with recurrent/persistent asthma symptoms. One hundred and sixty children (12-47 months) were randomised into this multicentre, double-blind, placebo-controlled, parallel-group study, and treated with either FP (100 microg bd) or placebo (2 puffs bd), both administered by metered-dose-inhaler and Babyhaler for 12 weeks. The primary endpoint was percentage of symptom-free 24h periods. Over weeks 1-12, FP-treated patients had significantly more percentage symptom-free 24-h periods compared with placebo (odds ratio 0.53; 95% CI 0.29-0.95; P = 0.035). Relative to baseline, where all patients were symptomatic for at least 21/28 days of the run-in, the improvement equated to one additional symptom-free 24 h period per week. FP patients also had a significantly higher percentage of 24 h periods with no wheeze or cough, the odds ratio for treatment difference corresponding to two additional wheeze-free and one additional cough-free periods per week. FP was well-tolerated, with similar reported adverse events in both groups. Urinary cortisol-creatinine ratio was slightly decreased among FP patients after 12 weeks, but with no clinical correlates. FP is effective for the treatment of chronic persistent asthma symptoms in very young children.