People born with a congenital immunodeficiency cannot adequately defend themselves against invading pathogens and are therefore particularly susceptible to infectious diseases. The first symptoms, usually infections, can occur at any time during a person's life – from birth to late adulthood.
But how many people does this affect in Germany? Which of the approximately 200 known forms of immunodeficiency do they suffer from? And how can they be treated more effectively? To answer such questions, data have been collected from patients in the German National Registry for Primary Immunodeficiencies (PID-NET Registry) since 2009.
Data analyzed and published
Prof. Bodo Grimbacher, MD, and his team have analyzed the data collected from documented centers in the PID-NET Registry and have now published it in the scientific journal Frontiers in Immunology. Grimbacher works at the Center for Chronic Immunodeficiency (CCI) of the University Hospital Freiburg and is a member of the Cluster of Excellence CIBSS – Centre for Integrative Biological Signalling Studies at the University of Freiburg and the Cluster of Excellence RESIST – Resolving Infection Susceptibility at the Hannover Medical School (MHH).
2,300 people in Germany with congenital immunodeficiency.
The team's study found that in Germany, at least 2.7 of every 100,000 citizens have congenital immunodeficiency, or about 2,300 people. Most of them have too few functioning antibodies, while others have what is known as immune dysregulation, often an autoimmune disease. "The results will ultimately lead to faster diagnoses and better treatments," Grimbacher says. The data in the publication refer to July 2017, and the number of registered patients has since risen to more than 3,200.
Different symptoms
"The analysis shows the wide range of symptoms with which patients with congenital disorders of the immune system can become ill. This is also reflected in the great importance of this disease, which accounts for more than 50 percent of the patients in the Center for Rare Diseases (ZSE) at MHH," says Prof. Reinhold E. Schmidt, Director of the MHH Department of Immunology and Rheumatology.
Original publication
El-Helou, S.M., Biegner, A., Bode, S., Ehl, S.R., …, Grimbacher, B. (2019). The German National Registry of Primary Immunodeficiencies (2012–2017). In: Front Immunol. DOI: https://doi.org/10.3389/fimmu.2019.01272