In the total cohort, the median delay for agammaglobulinaemias from 1991 to 2010 lay between 0·8 and 1·4, and between 1·9 and 2·2 for IgG subclass deficiency. In sIgA deficiency, the median delay lay between 1 and 1·8 years. WAS had a median delay between 0·4 and 0·6 years, AT between 1·8 and 3 years, DGS between 0·2 and 0·3 years and CGD between 0·6 and 1·4 years. SCID had a very short median delay of 0·1 to 0·2 years. Details on therapy were reported for 10 091 (81·8%) of the living patients. Of these, 4555 patients (45·1%) received immunoglobulin replacement, which makes it the most frequently reported long-term medication. A total of 3332 patients (73·2%) received immunoglobulins find more intravenously, while
it was administered subcutaneously in 1217 patients (26·7%). Twelve patients (0·3%) received intramuscular immunoglobulins. Six patients received both intravenous and subcutaneous treatment, which explains why the numbers total more than 100%. The
next most frequently reported medication concerns antibiotics (both prophylactic and therapeutic), which were given in 2703 patients (26·8%). Other frequently reported medications are steroids (548 patients, 5·4%), anti-fungals (509 patients, 5%), bronchodilators (275 patients, 2·7%) Fluorouracil research buy and immunosuppressants (271 patients, 2·7%); 809 patients (8%) had received a stem cell transplant; and 2375 patients (23·5%) had neither received a stem cell transplant nor were they receiving any long-term medication. Since we last published
data extracted from the ESID database in September 2008, the number of registered patients has almost doubled from 7430 to 13 708 patients. The distribution of patients with respect to the single PID entities has remained relatively stable since 2008. CVID continues to represent more than 20% of all registered patients. SIgA deficiency has replaced IgG subclass deficiency as the ALOX15 second most frequent entity. This is due mainly to the fact that this disease is reported very frequently in Spain and Hungary, countries that joined the ESID database after 2008. Most individuals with sIgA deficiency are free of infections [19], but are still included in the current ESID diagnostic criteria for PID. However, many centres obviously only report patients presenting with clinical manifestations, which means that reporting of this disease is extremely skewed. The prevalence numbers we calculated also differ strongly between countries. However, with 3240 living patients documented in the heterogeneous population of France, the overall prevalence of PID will not be less than 5:100 000. In general, the prevalence and incidence numbers produced from our data collection have to be interpreted with great caution. They can be seen as an indicator towards the actual numbers that would be calculated if the documentation was 100% complete. We believe that the differences we observed between countries and periods can most probably be attributed to under-reporting.