Hernandez-Trujillo MD C Merck Claritin Council Member; Baxter Advisory Group, Speaker and IFIR attendee; CSL Speaker. KPT276 KPT276 initial levels of IVIg doses, dosing intervals, routine use of prophylactic antibiotics, perceptions of the usefulness of subcutaneous immunoglobulin therapy (SCIg) and of the risk to patients’ health of policies adopted by health-care funders. Differences in practice were recognized and are discussed in terms of methods of health-care provision, which suggest future studies for ensuring continuation of appropriate levels of immunoglobulin replacement therapies. = 0057). Hyper-IgM (HIGM) syndrome presented a more dramatic difference, where 929% of ESID respondents recommended use of IVIg to treat the majority of these patients, whereas only 51% of general AAAAI respondents agreed ( 0001). These differences were not apparent when ESID and focused AAAAI respondents were compared (Fig. 1). In addition, ESID respondents recommended IVIg more frequently than general AAAAI respondents for severe combined immune deficiency (SCID) ( 0001), whereas the responses of the focused AAAAI respondents were statistically indistinguishable from those of ESID. The differences were largely the same as those recognized previously between the general and focused AAAAI users [5]. These findings are likely to indicate a need for increased awareness of practice KPT276 parameters and guidelines for the treatment of PID among subspecialists who divide their effort among immunology and other disciplines, as well as increased education in PID. A substantial proportion of general AAAAI users practice in a community-based setting that further distinguishes this group from ESID, and creates a potentially unique set of educational requires and difficulties. Open in a separate windows Fig. 1 Recommendation of immunoglobulin replacement for specific main immunodeficiencies. (a) Percentage of immunologists recommending intravenous immunoglobulin (IVIg) for more than 50% of patients with the specific main immunodeficiency disease (PID) outlined. (b) Percentage of immunologists recommending IVIg for at least some (5C50%) patients with the outlined diagnosis. = 0012). This may reflect a lack of clarity regarding the questionnaire, as definitions, KPT276 and therefore treatment implications, of IgAD with IgGSD and IgGSD alone vary between countries and continents. Dosing and infusion interval of IVIg therapies Interestingly, ESID respondents were equally likely (Fig. 2a) to recommending infusion frequencies of every 3 (456%) or 4 weeks (491%). Within the AAAAI membership, the vast majority (87%) recommended every 4 weeks as the most commonly recommended infusion interval for IVIg infusions for their patients [5]. This difference between ESID and both the AAAAI respondent groups was statistically significant ( 0001). This may reflect the greater use of self-infusion of IVIg by patients at home in Europe, which provides greater flexibility regarding infusion interval (although specific data do not exist to substantiate this hypothesis). More population-based databases need to be utilized to determine steps of end result in PID patients receiving IVIg every 3 every 4 weeks, as the efficacy of every 3-week dosing is currently unclear. Initial dosing of IVIg for PID patients naive to IVIg (Fig. 2b), however, showed strong agreement between all three subgroups (644C656%) that 400 mg/kg of IVIg should be used. Open in a separate windows Fig. 2 Intravenous immunoglobulin (IVIg) usage parameters. Comparison of current practice within immunologists of IVIg usage for treatment of antibody disorders. (a) Interval between IVIg infusions, (b) level of initial dosing of IVIg and (c) desired maintenance IgG trough levels (pre-infusion). = 0001). The converse was true: 269% of ESID respondents recommended higher trough levels of 751C900 mg/dl, whereas only 117% of general AAAAI respondents recommended this higher trough level ( 0001). Because IgG trough levels required to keep antibody deficiency patients infection-free have been identified as variable, spanning the normal range as in the general populace [7], the specific power of these values may switch with time. Perceptions regarding SCIg replacement therapy for PID SCIg replacement has been used as a therapy for PID in Europe Nes for more than 20 years [2]. SCIg replacement was only approved by the Food and Drug Administration (FDA) in the United States in 2006. Despite this difference in availability, ESID and focused KPT276 AAAAI respondents were similar in their responses, with the majority agreeing that SCIg replacement was equally as effective as IVIg in treating their PID patients (Fig. 3)..
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