Imunosuppressive Therapy

The keystone of treatment for EGPA remains corticosteroid therapy. The use of these various immunosuppressive medications was effective to treat symptoms of refractory EGPA and to minimize the development of relapses. If some defined prognostic risk factors (i.e., renal insufficiency, proteinuria >1gr/day, cardiomyopathy, central nervous system or gastrointestinal involvement) exist, the disease might be considered severe and should be treated more intensively with additional immunosuppressive therapy (16). Gendelman et al. review reported initially, all patients received corticosteroids, usually prednisone 1-2 mg/kg/day. Corticosteroids and additional immunosuppressive therapy were initially given in 10 out of 47 patients (21%). Disease relapse was reported in 23 out of 46 patients (50%) within half to 26 months. At relapse, 8 patients received corticosteroids monotherapy (35%) and 15 patients received additional immunosuppressive therapy (65%), usually cyclophosphamide, and in one case mycophenolate-mofetil. Eleftheriou et al. review reported treatment …show more content…
reported outcome in childhood EGPA is significantly worse with a 19% mortality rate in comparison with a 5% mortality rate in adult EGPA (3). Gendelman et al. reported the mortality of EGPA is substantial, 10 out of 47 patients (21%) died after disease duration of 2-26 months. Two patients died within short time when therapy was refused or stopped. Reported causes of death are: respiratory insufficiency (n =1), pulmonary abscess and sepsis (n=1), cardiac failure (n=3), cardiac arrest and severe myocarditis (n=1), intestinal perforation (n=2), intestinal perforation and septicemia (n=1), and gastrointestinal inflammation with necrosis and sepsis (n=1) (2). Eleftheriou et al. reported mortality rate was 15% (2/15); causes of death was presence of cardiomyopathy and high pediatric vasculitis damage index score (1). Early diagnosis and adequate treatment can lead to prevention of morbidity and