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The German national registry for primary immunodeficiencies (PID)
B Gathmann1, S Goldacker, M Klima
1Centre of Chronic Immunodeficiency, University Medical Center Freiburg and University of Freiburg, Freiburg, Germany. benjamin.gathmann@uniklinik-freiburg.de
Insights
Germany established its first national registry for primary immunodeficiencies (PID) in 2009. The registry now includes 1368 patients, with diagnostic delays persisting for conditions like common variable immunodeficiency (CVID).
Area of Science:
- Immunology
- Clinical Research
- Patient Registries
Background:
- Primary immunodeficiencies (PID) are a group of rare genetic disorders affecting the immune system.
- Establishing national patient registries is crucial for understanding PID epidemiology and improving patient care.
- Germany initiated its national PID registry in 2009 under the PID-NET consortium.
Purpose of the Study:
- To describe the establishment and initial findings of the German national PID registry.
- To analyze the demographic characteristics and diagnostic delays of PID patients in Germany.
- To assess the registry's growth and challenges in data collection.
Main Methods:
- Data from the European Database for Primary Immunodeficiencies (2004-2009) were incorporated.
- The national registry expanded from 7 to 21 centers between April 2009 and March 2012.
- Patient data, including clinical and genetic information, were collected and analyzed.
Main Results:
- The registry accumulated data on 1368 patients by March 2012, with 1232 alive.
- Significant gender differences in age distribution were observed in living patients.
- A median diagnostic delay of 4 years was noted for common variable immunodeficiency (CVID).
Conclusions:
- The German national PID registry has successfully aggregated substantial patient data.
- Persistent diagnostic delays, particularly for CVID, highlight a need for earlier detection strategies.
- The registry provides a valuable resource for research and improving outcomes for PID patients in Germany.
Abstract:
In 2009, a federally funded clinical and research consortium (PID-NET, http://www.pid-net.org) established the first national registry for primary immunodeficiencies (PID) in Germany. The registry contains clinical and genetic information on PID patients and is set up within the framework of the existing European Database for Primary Immunodeficiencies, run by the European Society for Primary Immunodeficiencies. Following the example of other national registries, a central data entry clerk has been employed to support data entry at the participating centres. Regulations for ethics approvals have presented a major challenge for participation of individual centres and have led to a delay in data entry in some cases. Data on 630 patients, entered into the European registry between 2004 and 2009, were incorporated into the national registry. From April 2009 to March 2012, the number of contributing centres increased from seven to 21 and 738 additional patients were reported, leading to a total number of 1368 patients, of whom 1232 were alive. The age distribution of living patients differs significantly by gender, with twice as many males than females among children, but 15% more women than men in the age group 30 years and older. The diagnostic delay between onset of symptoms and diagnosis has decreased for some PID over the past 20 years, but remains particularly high at a median of 4 years in common variable immunodeficiency (CVID), the most prevalent PID.
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