MARIAN ESCOBAR PALAZON, SpainHOSPITAL GREGORIO MARAÑON HOSPITAL DE DÍA INMUNOLOGÍA
Presenter Of 1 Presentation
FACILITATED SUBCUTANEOUS IMMUNOGLOBULIN THERAPY IN A PATIENT WITH SEVERE IGG HYPERCATABOLISM
Background and Aims
Background: Having a good life quality (QoL) is indispensable in patients with primary immunodeficiency (PID), because the lack of it, could affect negatively the patient’s life. IgG replacement therapy in these patients by use of subcutaneous immunoglobulin (SCIG) has good results in maintaining optimal immunoglobulin G (IgG) levels.
Aim: In a patient with severe IgG hyper catabolism facilitated subcutaneous immunoglobulin (FSCIG) therapy was administrated and health related QoL evaluated.
Methods: 35 year-old common variable immunodeficiency (CVID) patient. History of recurrent infections treated with intravenous immunoglobulin (IVIG). Due to protein hyper catabolism she couldn’t maintain optimal IgG levels so it was changed to combined therapy (IVIG 40 g/21 days + SCIG 8g/week; 2g/kg/month). The patient lives in a village 50 km far from the hospital and with a complicated familial environment, requiring psychological support. Due to the important deterioration of QoL, the treatment was changed to FSCIG, with a goal of maintaining IgG through above 400 mg/dL, in absence of recurrent infections, with a lower IgG dose. FSCIG was started at a dose of 25g/15 days (1g/kg/month). QoL was evaluated with SF-36 v.2.0.
Results: During a 4 month follow-up, after self-administered FSCIG at home, maintained IgG levels were 513 mg/dL. According to SF-36 improvement of health related QoL was detected in 21 of 36 items. There were no new infectious complications. FSCIG was well tolerated.
Conclusion: The use of FSCIG is a possible alternative in highly IgG hyper catabolic patients, improving the QoL, maintaining acceptable IgG through levels with lower doses.